Unmasking the Iron Veil: A Pediatric Case Report of Evans Syndrome Complicated by Iron Deficiency Anemia

Unmasking the Iron Veil: A Pediatric Case Report of Evans Syndrome Complicated by Iron Deficiency Anemia | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Unmasking the Iron Veil: A Pediatric Case Report of Evans Syndrome Complicated by Iron Deficiency Anemia Ahmadreza Ahmadi, Parnian Ahmadvand, Mohammad Hossein Salimian, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7161064/v1 This work is licensed under a CC BY 4.0 License Status: Under Review Version 1 posted 10 You are reading this latest preprint version Abstract Purpose To describe a pediatric case of Evans syndrome complicated by concurrent iron deficiency anemia that presented diagnostic challenges and highlight the importance of comprehensive hematologic evaluation in pediatric cytopenias. Methods We report a 14-year-old female presenting with a 2-week history of petechiae, pallor, and fatigue following an upper respiratory infection. Laboratory evaluation included complete blood count, direct antiglobulin test (DAT), comprehensive metabolic panel, iron studies, peripheral blood smear examination, and bone marrow biopsy. Treatment response to intravenous immunoglobulin (IVIG) and corticosteroids was monitored over 20 months. Results Initial findings revealed severe thrombocytopenia (48 × 10³/µL), Coombs-positive autoimmune hemolytic anemia (hemoglobin 8.1 g/dL, DAT IgG 3+), and concurrent iron deficiency anemia (ferritin 3 ng/mL). Peripheral blood smear demonstrated microcytic hypochromic anemia with spherocytes, dacrocytes, and thrombocytopenia with giant platelets. Physical examination revealed splenomegaly. Bone marrow biopsy showed normocellular marrow with erythroid hyperplasia and depleted iron stores, excluding primary bone marrow pathology. IVIG therapy (1 g/kg × 2 days) failed to improve platelet counts, but oral prednisone (2 mg/kg/day) achieved complete hematologic remission by day 24 (platelets 581 × 10³/µL, hemoglobin 11.9 g/dL). Iron supplementation corrected the deficiency. At 20-month follow-up, sustained hematologic remission was maintained (hemoglobin 13.8 g/dL, platelets 174 × 10³/µL), though elevated TSH (7 µIU/mL) indicated steroid-related endocrine effects. Conclusion Evans syndrome can be masked by concurrent iron deficiency anemia in pediatric patients, emphasizing the need for comprehensive autoimmune evaluation including DAT testing in children presenting with cytopenias. Corticosteroids remain effective first-line therapy, but long-term monitoring for steroid-related complications is essential. Evans syndrome iron deficiency anemia pediatric autoimmune hemolytic anemia immune thrombocytopenia corticosteroids Figures Figure 1 Figure 2 Figure 3 INTRODUCTION Evans syndrome (ES), first described in 1951, is a rare autoimmune disorder marked by immune thrombocytopenia (ITP) and Coombs-positive autoimmune hemolytic anemia (AIHA), affecting approximately 1–2 per 100,000 individuals annually [ 1 – 3 ]. In children, ES accounts for 5–15% of cytopenias, with relapse rates exceeding 50% and origins in immune dysregulation [ 3 , 4 ]. ES manifests with petechiae, hemolysis, and splenomegaly, requiring Coombs testing for confirmation [ 5 ]. In resource-limited settings, diagnostic delays are frequent, as iron deficiency anemia (IDA) often masks underlying autoimmunity [ 6 ]. Co-presentation with IDA adds diagnostic complexity [ 7 ]. First-line treatments such as corticosteroids and IVIG achieve remission in 60–70% of cases, but refractory cases may require rituximab or tailored steroid regimens to protect growth and minimize toxicity [ 8 , 9 ]. This report presents a 14-year-old girl whose ES, masked by IDA, highlights diagnostic challenges in pediatric cytopenias. CASE PRESENTATION A 14-year-old girl presented with a two-week history of petechiae, fatigue, pallor, and recent upper respiratory infection. Family history was negative for autoimmune disorders, hematologic malignancies, hereditary anemias, bleeding disorders, or consanguinity. Social history revealed iron-poor diet and no exposure to cytopenia-inducing medications, recent vaccinations, or blood transfusions. Physical examination revealed stable vital signs (temperature 36.8°C, heart rate 92 bpm, blood pressure 110/70 mmHg, respiratory rate 18/min). The patient appeared pale but alert and cooperative. Examination revealed pale conjunctivae, grade 2/6 systolic flow murmur consistent with anemia, and no lymphadenopathy. Abdominal examination demonstrated splenomegaly (palpable 3 cm below the left costal margin) with no hepatomegaly or abdominal masses. Skin examination revealed widespread petechiae on the trunk and extremities with no purpura, ecchymoses, or jaundice. Neurological examination was normal with no focal deficits. Laboratory findings confirmed Coombs-positive AIHA (hemoglobin 8.1 g/dL, MCV 61.4 fL, DAT IgG 3+, C3d 1+), thrombocytopenia (48 ×10^3/µL), IDA (ferritin 3 ng/mL), and low-normal folate (9.2 ng/mL), with normal B12 (823.0 pg/mL) (Table 1 ). Her platelet count, initially 174 ×10^3/µL prior to referral, fell to 48 ×10^3/µL upon hematology review, prompting IVIG (1 g/kg for two days). Evans syndrome was confirmed by the presence of simultaneous direct Coombs-positive autoimmune hemolytic anemia and immune thrombocytopenia [ 5 ]. Peripheral blood smear revealed microcytic hypochromic anemia, thrombocytopenia with giant platelets, and red cell abnormalities (spherocytes, dacrocytes, elliptocytes, Rouleaux formation, nucleated red blood cells), supporting autoimmune hemolysis and cytopenias (Fig. 1 ). Ultrasound showed a spleen measuring 138 ×40 mm, with a normal-sized liver. Table 1 Autoimmune and Biochemical Profile Test Result Reference Range Implication Direct Coombs (IgG/C3d) 3+/1+ → Negative (day 10) Negative Confirmed autoimmune hemolysis, normalized with treatment Reticulocyte Count (%) 2.0% (day 3), 2.97% (day 10) 0.5–2.5% Appropriate reticulocytic response to hemolysis Reticulocyte Production Index 0.7 (day 3), 1.48 (day 10) > 2.0 Low RPI indicating inadequate marrow response Ferritin (ng/mL) 3.0 → 21.1 (20 months) 15–200 ng/mL Severe iron deficiency, improved with supplementation ANA (Hep2) 1:80 titer < 1:40 Weak autoimmunity, no systemic lupus Anti-dsDNA (IU/mL) 16.12 < 30 IU/mL Ruled out lupus-associated Evans syndrome Alkaline Phosphatase (U/L) 416 → 253 44–147 U/L Persistently elevated, steroid-induced bone turnover TSH (µIU/mL) 2.84 → 7.0 0.4–4.0 µIU/mL Elevated, steroid-related thyroid dysfunction Vitamin D3 (ng/mL) 21.1 30–100 ng/mL Deficiency requiring supplementation Vitamin B12 (pg/mL) 823.0 200–900 pg/mL Normal Folate (ng/mL) 9.2 3.0–20.0 ng/mL Low-normal The bone marrow was normocellular with overall cellularity of 70–80%. Differential count revealed myeloid lineage 49%, erythroid lineage 22% with mild megaloblastic changes, and lymphocytes 15% with no excess blasts (< 1%). Myeloid-to-erythroid ratio was 1.4:1 (normal range 2–4:1), indicating relative erythroid hyperplasia consistent with hemolytic anemia. Megakaryocytes were adequate in number with normal morphology, ruling out thrombocytopenic purpura of central origin. No dysplastic changes, abnormal cells, or malignant infiltration were identified. Iron stores were markedly depleted, supporting concurrent iron deficiency anemia. These findings excluded primary bone marrow pathology and supported peripheral destruction of formed blood elements. Initial treatment with IVIG (1 g/kg/day for 2 consecutive days, total dose 2 g/kg) was ineffective to improve platelet counts (platelets remained at 30 ×10^3/µL at 48 hours post-completion) (Fig. 2 ). Prednisone was initiated on Day 3 at 2 mg/kg/day orally, continued at full dose for 1 month until complete hematologic remission was achieved (platelets 581 ×10³/µL, hemoglobin 11.9 g/dL by Day 24), then gradually tapered to discontinuation following sustained complete blood count normalization (Table 2 ) (Fig. 3 ). Concurrent supportive therapy included oral iron supplementation (3 mg/kg/day) starting Day 10 for 3 months and calcium supplementation (400 mg/day) initiated Day 10 and continued long-term for steroid-induced bone protection (Table 3 ). Table 2 Serial CBC Trends Parameter Day 1 Day 3 Day 10 Day 24 Day 34 20-month follow-up Hemoglobin (g/dL) 8.1 8.2 8.9 11.9 12.6 13.8 Platelets (×10^3/µL) 48 30 45 581 250 174 WBC (×10^3/µL) 8.9 9.08 9.02 19.21 15.63 6.6 MCV (fL) 61.4 63 63.7 70.4 73.2 82.5 Ferritin (ng/mL) 3 < 1.5 < 1.5 12.8 18.3 21.1 Table 3 Treatment Response Intervention Dose/Route Timeline Outcome IVIG 1 g/kg/day × 2 days (total 2 g/kg) Days 1–2 No response (platelets 30 ×10^3/µL at 48h) Prednisone 2 mg/kg/day per os (PO) × 1 month, then gradual taper to discontinuation after remission From day 3 onward Platelets 581 ×10^3/µL by day 24 Oral Iron 3 mg/kg/day PO Day 10–3 months Ferritin 21.1 ng/mL at 20 months Calcium Supplement 400 mg/day PO From day 10 onward Bone protection Final Diagnosis: 1. ES (AIHA + ITP); 2. IDA. DISCUSSION: Unraveling the Blood’s Silent Symphony This 14-year-old girl’s journey through Evans syndrome (ES) and iron deficiency anemia (IDA) reflects hidden clinical challenges, framed as follows: 4.1 Diagnostic Pitfalls: The Iron Veil’s Masquerade Severe IDA (ferritin 3 ng/mL) cloaked the autoimmune storm, an uncommon but critical twist in pediatric ES [ 7 ]. Iron deficiency may worsen hemolysis by impairing red cell production, while folate levels were low-normal (9.2 ng/mL, normal range 3.0–20.0 ng/mL), ruling out megaloblastic anemia as a contributor [ 10 ]. This underscores the need for routine Coombs testing and nutritional screening in pediatric cytopenias [ 5 ]. 4.2 Differential Diagnosis: Through The Looking Glass of Evans Systematic exclusion of alternative etiologies included ruling out primary bone marrow pathology (normocellular aspirate, M/E ratio 1.4, blasts < 1%) and hereditary disorders (negative family history, isolated splenomegaly). While nutritional deficiencies contributed, positive DAT and immunosuppression response confirmed autoimmune etiology [ 13 , 14 ]. 4.3 First-Line Clarity: Steroids Outshine IVIG in Treatment Response IVIG (1 g/kg ×2 days) failed to improve platelets (30 ×10^3/µL), but prednisone achieved normalization—supporting steroids as first-line therapy in pediatric ES [ 6 , 8 , 4 ]. IVIG nonresponsiveness highlights the need for rapid escalation to steroids. 4.4 Long-Term Monitoring: Healing’s Echoes on Fragile Bones At 20 months, hematologic remission held strong (hemoglobin 13.8 g/dL, platelets 174 ×10^3/µL), but elevated TSH (7 µIU/mL), and ALP (decreased from 416 U/L to 253 U/L) reflect steroid-related effects on endocrine and renal function. Persistent vitamin D deficiency (21.1 ng/mL) heightens bone health risks, necessitating supplementation and monitoring [ 12 ]. These findings call for vigilant long-term follow-up in pediatric ES patients on steroids [ 11 ]. 4.5 Immunologic and Nutritional Vigilance—A Pediatric Imperative The co-occurrence of ES and IDA demands vigilance through dietary assessment, iron monitoring, and tailored immunosuppression to minimize complications. CONCLUSION In this 14-year-old girl, Evans syndrome masked by iron deficiency anemia achieved sustained hematologic remission at 20 months (hemoglobin 13.8 g/dL, platelets 174 × 10^3/µL) following prednisone therapy and iron supplementation. However, elevated TSH (7 µIU/mL) and persistent ALP elevation (253 U/L) warrant continued endocrine and bone health surveillance. Clinicians should screen for IDA and autoimmunity in pediatric cytopenias, prioritize corticosteroids for ES treatment, and monitor long-term steroid complications. Declarations 6.1 Acknowledgements We are grateful to the patient and her family for their consent and to the Alborz University of Medical Sciences research team for their support. We also acknowledge the laboratory staff for technical contribution. 6.2 Author Contributions Ahmadreza Ahmadi: Conceptualization (lead); Investigation (equal); Writing – original draft (lead); Writing – review & editing (lead); Data curation (lead); Formal analysis (lead); Methodology (lead); Project administration (lead). Parnian Ahmadvand: Conceptualization (supporting); Investigation (supporting); Writing – original draft (supporting); Writing – review & editing (supporting); Data curation (supporting); Project administration (supporting). Mohammad Hossein Salimian: Investigation (lead); Writing – original draft (supporting); Writing – review & editing (supporting); Formal analysis (supporting). Yazdan-Mehr Firouzi: Investigation (supporting); Writing – review & editing (supporting); Methodology (supporting); Literature review (supporting). All authors approved the final manuscript. 6.3 Funding This research received no specific grant from funding agencies in the public, commercial, or not-for-profit sectors. 6.4 Conflict of Interest The authors declare no competing interests, financial or otherwise, relevant to this work. 6.5 Ethical Compliance This retrospective case report was exempt from full ethical approval by the Alborz University of medical Sciences Institutional Review Board (IRB) due to its non-interventional nature. The study received IRB acknowledgment and adhered to the 1964 Helsinki Declaration and its amendments. Clinical trial number: not applicable. 6.6 Patient Consent Written informed consent was obtained from the patient’s legal guardian for publication of de-identified clinical data and images. 6.7 Consent to Publish Written informed consent was obtained from the patient’s legal guardian for the publication of this case report and any accompanying de-identified clinical data and images. 6.8 Consent to Participate Not applicable. This is a retrospective case report based on a review of existing medical records and did not involve any new intervention or participation from the patient. 7. DATA AVAILABILITY STATEMENT De-identified patient data and laboratory results are available from the corresponding author on reasonable request. Peripheral blood smear images are included as figures. References Aladjidi N, Fernandes H, Leblanc T, Vareliette A, Rieux-Laucat F, Bertrand Y, et al. Evans syndrome in children: long-term outcome in a prospective French national observational cohort. Front Pediatr. 2015;3:79. doi:10.3389/fped.2015.00079 Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951;87(1):48-65. doi:10.1001/archinte.1951.03810010058005 Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018;9:171-84. doi:10.2147/JBM.S176144 Pincez T, Fernandes H, Leblanc T, Michel G, Barlogis V, Bertrand Y, et al. Long term follow-up of pediatric-onset Evans syndrome: broad immunopathological manifestations and high treatment burden. Haematologica. 2022;107(2):457-66. doi:10.3324/haematol.2020.271106 Fattizzo B, Marchetti M, Michel M, Cantoni S, Frederiksen H, Giordano G, et al. Diagnosis and management of Evans syndrome in adults: first consensus recommendations. Lancet Haematol. 2024;11(8):e617-e628. doi:10.1016/s2352-3026(24)00144-3 Michel M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167-72. doi:10.1182/blood-2009-04-215368 Powers JM, Buchanan GR. Diagnosis and management of iron deficiency anemia. Hematol Oncol Clin North Am. 2014;28(4):729-45. doi:10.1016/j.hoc.2014.04.007 Norton A, Roberts I. Management of Evans syndrome. Br J Haematol. 2006;132(2):125-37. doi:10.1111/j.1365-2141.2005.05809.x Zecca M, Nobili B, Ramenghi U, Perrotta S, Amendola G, Rosito P, et al. Rituximab for the treatment of refractory autoimmune hemolytic anemia in children. Blood. 2003;101(10):3857-61. doi:10.1182/blood-2002-11-3547 Camaschella, C. (2015). Iron-deficiency anemia. New England Journal of Medicine, 372(19), 1832-1843. doi:10.1056/NEJMra1401038 Audia S, Mahévas M, Nivet M, Ouandji S, Ciudad M, Bonnotte B. Immune thrombocytopenia: recent advances in pathogenesis and treatments. Hemasphere. 2021;5(6):e574. doi:10.1097/HS9.0000000000000574 Gahr K, Sommers N, Bostrom B. Bone mineral metabolism during chemotherapy in childhood acute lymphoblastic leukemia: unexpected vitamin D deficiency from induction corticosteroids in acute lymphoblastic leukemia. J Pediatr Hematol Oncol. 2021;43(6):240-1. doi:10.1097/MPH.0000000000002028 Miano M, Guardo D, Grossi A, Palmisani E, Fioredda F, Terranova P, et al. Underlying inborn errors of immunity in patients with Evans syndrome and multilineage cytopenias: a single-centre analysis. Front Immunol. 2022;13:869033. doi:10.3389/fimmu.2022.869033 Lechner K, Jäger U. How I treat autoimmune hemolytic anemias in adults. Blood. 2010;116(11):1831-8. doi:10.1182/blood-2010-03-259325 Additional Declarations No competing interests reported. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7161064","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":509871932,"identity":"ccb13804-00fd-49d3-982f-ebf08f78ec43","order_by":0,"name":"Ahmadreza Ahmadi","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA50lEQVRIie3RsarCMBSA4VOEupzQtRKpr9BSqIODr6I4uKSO4nApBUE3fQJfI3PkgF3q3tnBSUURHC83i8hdUt0c8kGWkJ+QHADL+k6uXg30AF9b6q2klX+aQKiw5tyTlzeOh+qn146LPd2ZyMBbKodmhsRXbjcWuzEm5WQQM0nglwPYlqZrFCRcuISJEuGISf2KCmCbG4qOaj64+CWM16eQmMygU5foVyc8XRCGvojmTOp/qEsiwilPV2P0q8vI2UjCqBzmxiQolpKLR6/vrVO6nmUWBAXRzZToifynp+MYA8uyLOsNfy/SSXlfN7mhAAAAAElFTkSuQmCC","orcid":"","institution":"Iran University of Medical Sciences","correspondingAuthor":true,"prefix":"","firstName":"Ahmadreza","middleName":"","lastName":"Ahmadi","suffix":""},{"id":509871933,"identity":"f8bb40c5-cf88-4930-8b37-6073e942354a","order_by":1,"name":"Parnian Ahmadvand","email":"","orcid":"","institution":"Alborz University of Medical Sciences","correspondingAuthor":false,"prefix":"","firstName":"Parnian","middleName":"","lastName":"Ahmadvand","suffix":""},{"id":509871934,"identity":"afda78f9-3c57-4ba9-bae9-c417f1937447","order_by":2,"name":"Mohammad Hossein Salimian","email":"","orcid":"","institution":"Kashan University of Medical Sciences","correspondingAuthor":false,"prefix":"","firstName":"Mohammad","middleName":"Hossein","lastName":"Salimian","suffix":""},{"id":509871935,"identity":"77b3fb8f-65ef-4869-9a73-d91f08c007cc","order_by":3,"name":"Yazdan-Mehr Firouzi","email":"","orcid":"","institution":"International Students’ Association (TUMS-ISA), Tehran University of Medical Sciences","correspondingAuthor":false,"prefix":"","firstName":"Yazdan-Mehr","middleName":"","lastName":"Firouzi","suffix":""}],"badges":[],"createdAt":"2025-07-19 00:53:11","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7161064/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7161064/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":90881750,"identity":"40e353ff-ae99-499a-af10-5184c687b05f","added_by":"auto","created_at":"2025-09-09 09:48:17","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":245803,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003ePeripheral blood smear (Wright-Giemsa stain, 100 × magnification) revealing microcytic hypochromic anemia and thrombocytopenia, with giant platelets. Prominent red blood cell morphological abnormalities include Rouleaux formation, spherocytes, dacrocytes (teardrop cells), and elliptocytes, collectively indicative of hemolytic stress and immune-mediated cytopenias in this Evans syndrome case. These findings align with the triad of autoimmune hemolysis and thrombocytopenia characteristic of the disorder, with nucleated red blood cells (NRBCs) further supporting hemolytic stress.\u003c/em\u003e\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-7161064/v1/a961da7a029cda951f15dee5.png"},{"id":90881299,"identity":"57199bf5-ff0a-4e10-8cab-84f98d0f6918","added_by":"auto","created_at":"2025-09-09 09:40:13","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":123498,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eBar graph illustrating platelet response to IVIG and prednisone in pediatric Evans syndrome, demonstrating marked improvement following prednisone therapy.\u003c/em\u003e\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-7161064/v1/89d62b71eeb87b4f93131da6.png"},{"id":90881746,"identity":"decbd7e7-ca71-4632-8d9d-17f5eaf17169","added_by":"auto","created_at":"2025-09-09 09:48:14","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":127851,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eLine graph showing hematologic trends (hemoglobin, platelets, white blood cell count) over 20 months in a pediatric patient with Evans syndrome, highlighting remission following prednisone treatment.\u003c/em\u003e\u003c/p\u003e","description":"","filename":"floatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-7161064/v1/6237214a69c2c4a4b8750b44.png"},{"id":90881772,"identity":"e9142176-fd75-498c-a9af-526b1dfdb680","added_by":"auto","created_at":"2025-09-09 09:48:21","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1238993,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7161064/v1/9f095e11-0a5d-4b41-8cfd-dba9e9d3c1fd.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003eUnmasking the Iron Veil: A Pediatric Case Report of Evans Syndrome Complicated by Iron Deficiency Anemia\u003c/p\u003e","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003eEvans syndrome (ES), first described in 1951, is a rare autoimmune disorder marked by immune thrombocytopenia (ITP) and Coombs-positive autoimmune hemolytic anemia (AIHA), affecting approximately 1\u0026ndash;2 per 100,000 individuals annually [\u003cspan additionalcitationids=\"CR2\" citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. In children, ES accounts for 5\u0026ndash;15% of cytopenias, with relapse rates exceeding 50% and origins in immune dysregulation [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. ES manifests with petechiae, hemolysis, and splenomegaly, requiring Coombs testing for confirmation [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. In resource-limited settings, diagnostic delays are frequent, as iron deficiency anemia (IDA) often masks underlying autoimmunity [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Co-presentation with IDA adds diagnostic complexity [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. First-line treatments such as corticosteroids and IVIG achieve remission in 60\u0026ndash;70% of cases, but refractory cases may require rituximab or tailored steroid regimens to protect growth and minimize toxicity [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. This report presents a 14-year-old girl whose ES, masked by IDA, highlights diagnostic challenges in pediatric cytopenias.\u003c/p\u003e"},{"header":"CASE PRESENTATION","content":"\u003cp\u003eA 14-year-old girl presented with a two-week history of petechiae, fatigue, pallor, and recent upper respiratory infection. Family history was negative for autoimmune disorders, hematologic malignancies, hereditary anemias, bleeding disorders, or consanguinity.\u003c/p\u003e\u003cp\u003eSocial history revealed iron-poor diet and no exposure to cytopenia-inducing medications, recent vaccinations, or blood transfusions.\u003c/p\u003e\u003cp\u003ePhysical examination revealed stable vital signs (temperature 36.8\u0026deg;C, heart rate 92 bpm, blood pressure 110/70 mmHg, respiratory rate 18/min). The patient appeared pale but alert and cooperative. Examination revealed pale conjunctivae, grade 2/6 systolic flow murmur consistent with anemia, and no lymphadenopathy. Abdominal examination demonstrated splenomegaly (palpable 3 cm below the left costal margin) with no hepatomegaly or abdominal masses. Skin examination revealed widespread petechiae on the trunk and extremities with no purpura, ecchymoses, or jaundice. Neurological examination was normal with no focal deficits.\u003c/p\u003e\u003cp\u003eLaboratory findings confirmed Coombs-positive AIHA (hemoglobin 8.1 g/dL, MCV 61.4 fL, DAT IgG 3+, C3d 1+), thrombocytopenia (48 \u0026times;10^3/\u0026micro;L), IDA (ferritin 3 ng/mL), and low-normal folate (9.2 ng/mL), with normal B12 (823.0 pg/mL) (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Her platelet count, initially 174 \u0026times;10^3/\u0026micro;L prior to referral, fell to 48 \u0026times;10^3/\u0026micro;L upon hematology review, prompting IVIG (1 g/kg for two days). Evans syndrome was confirmed by the presence of simultaneous direct Coombs-positive autoimmune hemolytic anemia and immune thrombocytopenia [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Peripheral blood smear revealed microcytic hypochromic anemia, thrombocytopenia with giant platelets, and red cell abnormalities (spherocytes, dacrocytes, elliptocytes, Rouleaux formation, nucleated red blood cells), supporting autoimmune hemolysis and cytopenias (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Ultrasound showed a spleen measuring 138 \u0026times;40 mm, with a normal-sized liver.\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eAutoimmune and Biochemical Profile\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"4\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTest\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eResult\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eReference Range\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eImplication\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eDirect Coombs (IgG/C3d)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3+/1+ \u0026rarr; Negative (day 10)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eNegative\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eConfirmed autoimmune hemolysis, normalized with treatment\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eReticulocyte Count (%)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2.0% (day 3), 2.97% (day 10)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e0.5\u0026ndash;2.5%\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eAppropriate reticulocytic response to hemolysis\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eReticulocyte Production Index\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e0.7 (day 3), 1.48 (day 10)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026gt;\u0026thinsp;2.0\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eLow RPI indicating inadequate marrow response\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eFerritin (ng/mL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3.0 \u0026rarr; 21.1 (20 months)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e15\u0026ndash;200 ng/mL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eSevere iron deficiency, improved with supplementation\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eANA (Hep2)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e1:80 titer\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026lt;\u0026thinsp;1:40\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eWeak autoimmunity, no systemic lupus\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eAnti-dsDNA (IU/mL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e16.12\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026lt;\u0026thinsp;30 IU/mL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eRuled out lupus-associated Evans syndrome\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eAlkaline Phosphatase (U/L)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e416 \u0026rarr; 253\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e44\u0026ndash;147 U/L\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003ePersistently elevated, steroid-induced bone turnover\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eTSH (\u0026micro;IU/mL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2.84 \u0026rarr; 7.0\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e0.4\u0026ndash;4.0 \u0026micro;IU/mL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eElevated, steroid-related thyroid dysfunction\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eVitamin D3 (ng/mL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e21.1\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e30\u0026ndash;100 ng/mL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eDeficiency requiring supplementation\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eVitamin B12 (pg/mL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e823.0\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e200\u0026ndash;900 pg/mL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eNormal\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eFolate (ng/mL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e9.2\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3.0\u0026ndash;20.0 ng/mL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eLow-normal\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eThe bone marrow was normocellular with overall cellularity of 70\u0026ndash;80%. Differential count revealed myeloid lineage 49%, erythroid lineage 22% with mild megaloblastic changes, and lymphocytes 15% with no excess blasts (\u0026lt;\u0026thinsp;1%). Myeloid-to-erythroid ratio was 1.4:1 (normal range 2\u0026ndash;4:1), indicating relative erythroid hyperplasia consistent with hemolytic anemia. Megakaryocytes were adequate in number with normal morphology, ruling out thrombocytopenic purpura of central origin. No dysplastic changes, abnormal cells, or malignant infiltration were identified. Iron stores were markedly depleted, supporting concurrent iron deficiency anemia. These findings excluded primary bone marrow pathology and supported peripheral destruction of formed blood elements.\u003c/p\u003e\u003cp\u003eInitial treatment with IVIG (1 g/kg/day for 2 consecutive days, total dose 2 g/kg) was ineffective to improve platelet counts (platelets remained at 30 \u0026times;10^3/\u0026micro;L at 48 hours post-completion) (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Prednisone was initiated on Day 3 at 2 mg/kg/day orally, continued at full dose for 1 month until complete hematologic remission was achieved (platelets 581 \u0026times;10\u0026sup3;/\u0026micro;L, hemoglobin 11.9 g/dL by Day 24), then gradually tapered to discontinuation following sustained complete blood count normalization (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e) (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). Concurrent supportive therapy included oral iron supplementation (3 mg/kg/day) starting Day 10 for 3 months and calcium supplementation (400 mg/day) initiated Day 10 and continued long-term for steroid-induced bone protection (Table\u0026nbsp;\u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eSerial CBC Trends\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"7\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u003cp\u003eParameter\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eDay 1\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDay 3\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eDay 10\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c5\"\u003e\u003cp\u003eDay 24\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c6\"\u003e\u003cp\u003eDay 34\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c7\"\u003e\u003cp\u003e20-month follow-up\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eHemoglobin (g/dL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e8.1\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e8.2\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003e8.9\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u003cp\u003e11.9\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c6\"\u003e\u003cp\u003e12.6\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c7\"\u003e\u003cp\u003e13.8\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003ePlatelets (\u0026times;10^3/\u0026micro;L)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e48\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e30\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003e45\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u003cp\u003e581\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c6\"\u003e\u003cp\u003e250\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c7\"\u003e\u003cp\u003e174\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eWBC (\u0026times;10^3/\u0026micro;L)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e8.9\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e9.08\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003e9.02\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u003cp\u003e19.21\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c6\"\u003e\u003cp\u003e15.63\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c7\"\u003e\u003cp\u003e6.6\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eMCV (fL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e61.4\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e63\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003e63.7\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u003cp\u003e70.4\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c6\"\u003e\u003cp\u003e73.2\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c7\"\u003e\u003cp\u003e82.5\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eFerritin (ng/mL)\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u0026lt;\u0026thinsp;1.5\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003e\u0026lt;\u0026thinsp;1.5\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u003cp\u003e12.8\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c6\"\u003e\u003cp\u003e18.3\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c7\"\u003e\u003cp\u003e21.1\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab3\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eTreatment Response\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"4\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u003cp\u003eIntervention\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eDose/Route\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eTimeline\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eOutcome\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eIVIG\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e1 g/kg/day \u0026times; 2 days (total 2 g/kg)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDays 1\u0026ndash;2\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eNo response (platelets 30 \u0026times;10^3/\u0026micro;L at 48h)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003ePrednisone\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2 mg/kg/day per os (PO) \u0026times; 1 month, then gradual taper to discontinuation after remission\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eFrom day 3 onward\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003ePlatelets 581 \u0026times;10^3/\u0026micro;L by day 24\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eOral Iron\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3 mg/kg/day PO\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eDay 10\u0026ndash;3 months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eFerritin 21.1 ng/mL at 20 months\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e\u003cb\u003eCalcium Supplement\u003c/b\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e400 mg/day PO\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003eFrom day 10 onward\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u003cp\u003eBone protection\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003eFinal Diagnosis: 1. ES (AIHA\u0026thinsp;+\u0026thinsp;ITP); 2. IDA.\u003c/p\u003e"},{"header":"DISCUSSION: Unraveling the Blood’s Silent Symphony","content":"\u003cp\u003eThis 14-year-old girl\u0026rsquo;s journey through Evans syndrome (ES) and iron deficiency anemia (IDA) reflects hidden clinical challenges, framed as follows:\u003c/p\u003e\u003cdiv id=\"Sec4\" class=\"Section2\"\u003e\u003ch2\u003e4.1 Diagnostic Pitfalls: The Iron Veil\u0026rsquo;s Masquerade\u003c/h2\u003e\u003cp\u003eSevere IDA (ferritin 3 ng/mL) cloaked the autoimmune storm, an uncommon but critical twist in pediatric ES [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Iron deficiency may worsen hemolysis by impairing red cell production, while folate levels were low-normal (9.2 ng/mL, normal range 3.0\u0026ndash;20.0 ng/mL), ruling out megaloblastic anemia as a contributor [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. This underscores the need for routine Coombs testing and nutritional screening in pediatric cytopenias [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e].\u003c/p\u003e\u003c/div\u003e\u003cdiv id=\"Sec5\" class=\"Section2\"\u003e\u003ch2\u003e4.2 Differential Diagnosis: Through The Looking Glass of Evans\u003c/h2\u003e\u003cp\u003eSystematic exclusion of alternative etiologies included ruling out primary bone marrow pathology (normocellular aspirate, M/E ratio 1.4, blasts\u0026thinsp;\u0026lt;\u0026thinsp;1%) and hereditary disorders (negative family history, isolated splenomegaly). While nutritional deficiencies contributed, positive DAT and immunosuppression response confirmed autoimmune etiology [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e\u003c/div\u003e\u003cdiv id=\"Sec6\" class=\"Section2\"\u003e\u003ch2\u003e4.3 First-Line Clarity: Steroids Outshine IVIG in Treatment Response\u003c/h2\u003e\u003cp\u003eIVIG (1 g/kg \u0026times;2 days) failed to improve platelets (30 \u0026times;10^3/\u0026micro;L), but prednisone achieved normalization\u0026mdash;supporting steroids as first-line therapy in pediatric ES [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. IVIG nonresponsiveness highlights the need for rapid escalation to steroids.\u003c/p\u003e\u003c/div\u003e\u003cdiv id=\"Sec7\" class=\"Section2\"\u003e\u003ch2\u003e4.4 Long-Term Monitoring: Healing\u0026rsquo;s Echoes on Fragile Bones\u003c/h2\u003e\u003cp\u003eAt 20 months, hematologic remission held strong (hemoglobin 13.8 g/dL, platelets 174 \u0026times;10^3/\u0026micro;L), but elevated TSH (7 \u0026micro;IU/mL), and ALP (decreased from 416 U/L to 253 U/L) reflect steroid-related effects on endocrine and renal function. Persistent vitamin D deficiency (21.1 ng/mL) heightens bone health risks, necessitating supplementation and monitoring [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. These findings call for vigilant long-term follow-up in pediatric ES patients on steroids [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e].\u003c/p\u003e\u003c/div\u003e\u003cdiv id=\"Sec8\" class=\"Section2\"\u003e\u003ch2\u003e4.5 Immunologic and Nutritional Vigilance\u0026mdash;A Pediatric Imperative\u003c/h2\u003e\u003cp\u003eThe co-occurrence of ES and IDA demands vigilance through dietary assessment, iron monitoring, and tailored immunosuppression to minimize complications.\u003c/p\u003e\u003c/div\u003e"},{"header":"CONCLUSION","content":"\u003cp\u003eIn this 14-year-old girl, Evans syndrome masked by iron deficiency anemia achieved sustained hematologic remission at 20 months (hemoglobin 13.8 g/dL, platelets 174 \u0026times; 10^3/\u0026micro;L) following prednisone therapy and iron supplementation. However, elevated TSH (7 \u0026micro;IU/mL) and persistent ALP elevation (253 U/L) warrant continued endocrine and bone health surveillance. Clinicians should screen for IDA and autoimmunity in pediatric cytopenias, prioritize corticosteroids for ES treatment, and monitor long-term steroid complications.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003e6.1 Acknowledgements \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe are grateful to the patient and her family for their consent and to the Alborz University of Medical Sciences research team for their support. We also acknowledge the laboratory staff for technical contribution.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e6.2 Author Contributions \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAhmadreza Ahmadi: Conceptualization (lead); Investigation (equal); Writing \u0026ndash; original draft (lead); Writing \u0026ndash; review \u0026amp; editing (lead); Data curation (lead); Formal analysis (lead); Methodology (lead); Project administration (lead).\u003c/p\u003e\n\u003cp\u003eParnian Ahmadvand: Conceptualization (supporting); Investigation (supporting); Writing \u0026ndash; original draft (supporting); Writing \u0026ndash; review \u0026amp; editing (supporting); Data curation (supporting); Project administration (supporting).\u003c/p\u003e\n\u003cp\u003eMohammad Hossein Salimian: Investigation (lead); Writing \u0026ndash; original draft (supporting); Writing \u0026ndash; review \u0026amp; editing (supporting); Formal analysis (supporting).\u003c/p\u003e\n\u003cp\u003eYazdan-Mehr Firouzi: Investigation (supporting); Writing \u0026ndash; review \u0026amp; editing (supporting); Methodology (supporting); Literature review (supporting).\u003c/p\u003e\n\u003cp\u003eAll authors approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003cstrong\u003e6.3 Funding \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis research received no specific grant from funding agencies in the public, commercial, or not-for-profit sectors.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e6.4 Conflict of Interest \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no competing interests, financial or otherwise, relevant to this work.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e6.5 Ethical Compliance \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis retrospective case report was exempt from full ethical approval by the Alborz University of medical Sciences Institutional Review Board (IRB) due to its non-interventional nature. The study received IRB acknowledgment and adhered to the 1964 Helsinki Declaration and its amendments. Clinical trial number: not applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e6.6 Patient Consent \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient\u0026rsquo;s legal guardian for publication of de-identified clinical data and images.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e6.7 Consent to Publish\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient\u0026rsquo;s legal guardian for the publication of this case report and any accompanying de-identified clinical data and images.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e6.8 Consent to Participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable. This is a retrospective case report based on a review of existing medical records and did not involve any new intervention or participation from the patient.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003cstrong\u003e7. DATA AVAILABILITY STATEMENT \u0026nbsp;\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eDe-identified patient data and laboratory results are available from the corresponding author on reasonable request. Peripheral blood smear images are included as figures.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eAladjidi N, Fernandes H, Leblanc T, Vareliette A, Rieux-Laucat F, Bertrand Y, et al. Evans syndrome in children: long-term outcome in a prospective French national observational cohort. Front Pediatr. 2015;3:79. doi:10.3389/fped.2015.00079\u003c/li\u003e\n\u003cli\u003eEvans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951;87(1):48-65. doi:10.1001/archinte.1951.03810010058005\u003c/li\u003e\n\u003cli\u003eJaime-P\u0026eacute;rez JC, Aguilar-Calder\u0026oacute;n PE, Salazar-Cavazos L, G\u0026oacute;mez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018;9:171-84. doi:10.2147/JBM.S176144\u003c/li\u003e\n\u003cli\u003ePincez T, Fernandes H, Leblanc T, Michel G, Barlogis V, Bertrand Y, et al. Long term follow-up of pediatric-onset Evans syndrome: broad immunopathological manifestations and high treatment burden. Haematologica. 2022;107(2):457-66. doi:10.3324/haematol.2020.271106\u003c/li\u003e\n\u003cli\u003eFattizzo B, Marchetti M, Michel M, Cantoni S, Frederiksen H, Giordano G, et al. Diagnosis and management of Evans syndrome in adults: first consensus recommendations. Lancet Haematol. 2024;11(8):e617-e628. doi:10.1016/s2352-3026(24)00144-3\u003c/li\u003e\n\u003cli\u003eMichel M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167-72. doi:10.1182/blood-2009-04-215368\u003c/li\u003e\n\u003cli\u003ePowers JM, Buchanan GR. Diagnosis and management of iron deficiency anemia. Hematol Oncol Clin North Am. 2014;28(4):729-45. doi:10.1016/j.hoc.2014.04.007\u003c/li\u003e\n\u003cli\u003eNorton A, Roberts I. Management of Evans syndrome. Br J Haematol. 2006;132(2):125-37. doi:10.1111/j.1365-2141.2005.05809.x\u003c/li\u003e\n\u003cli\u003eZecca M, Nobili B, Ramenghi U, Perrotta S, Amendola G, Rosito P, et al. Rituximab for the treatment of refractory autoimmune hemolytic anemia in children. Blood. 2003;101(10):3857-61. doi:10.1182/blood-2002-11-3547\u003c/li\u003e\n\u003cli\u003eCamaschella, C. (2015). Iron-deficiency anemia. New England Journal of Medicine, 372(19), 1832-1843. doi:10.1056/NEJMra1401038\u003c/li\u003e\n\u003cli\u003eAudia S, Mah\u0026eacute;vas M, Nivet M, Ouandji S, Ciudad M, Bonnotte B. Immune thrombocytopenia: recent advances in pathogenesis and treatments. Hemasphere. 2021;5(6):e574. doi:10.1097/HS9.0000000000000574\u003c/li\u003e\n\u003cli\u003eGahr K, Sommers N, Bostrom B. Bone mineral metabolism during chemotherapy in childhood acute lymphoblastic leukemia: unexpected vitamin D deficiency from induction corticosteroids in acute lymphoblastic leukemia. J Pediatr Hematol Oncol. 2021;43(6):240-1. doi:10.1097/MPH.0000000000002028\u003c/li\u003e\n\u003cli\u003eMiano M, Guardo D, Grossi A, Palmisani E, Fioredda F, Terranova P, et al. Underlying inborn errors of immunity in patients with Evans syndrome and multilineage cytopenias: a single-centre analysis. Front Immunol. 2022;13:869033. doi:10.3389/fimmu.2022.869033\u003c/li\u003e\n\u003cli\u003eLechner K, J\u0026auml;ger U. How I treat autoimmune hemolytic anemias in adults. Blood. 2010;116(11):1831-8. doi:10.1182/blood-2010-03-259325\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-pediatrics","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bped","sideBox":"Learn more about [BMC Pediatrics](http://bmcpediatr.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bped/default.aspx","title":"BMC Pediatrics","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Evans syndrome, iron deficiency anemia, pediatric, autoimmune hemolytic anemia, immune thrombocytopenia, corticosteroids","lastPublishedDoi":"10.21203/rs.3.rs-7161064/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7161064/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003ePurpose\u003c/h2\u003e\u003cp\u003eTo describe a pediatric case of Evans syndrome complicated by concurrent iron deficiency anemia that presented diagnostic challenges and highlight the importance of comprehensive hematologic evaluation in pediatric cytopenias.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e\u003cp\u003eWe report a 14-year-old female presenting with a 2-week history of petechiae, pallor, and fatigue following an upper respiratory infection. Laboratory evaluation included complete blood count, direct antiglobulin test (DAT), comprehensive metabolic panel, iron studies, peripheral blood smear examination, and bone marrow biopsy. Treatment response to intravenous immunoglobulin (IVIG) and corticosteroids was monitored over 20 months.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e\u003cp\u003eInitial findings revealed severe thrombocytopenia (48 \u0026times; 10\u0026sup3;/\u0026micro;L), Coombs-positive autoimmune hemolytic anemia (hemoglobin 8.1 g/dL, DAT IgG 3+), and concurrent iron deficiency anemia (ferritin 3 ng/mL). Peripheral blood smear demonstrated microcytic hypochromic anemia with spherocytes, dacrocytes, and thrombocytopenia with giant platelets. Physical examination revealed splenomegaly. Bone marrow biopsy showed normocellular marrow with erythroid hyperplasia and depleted iron stores, excluding primary bone marrow pathology. IVIG therapy (1 g/kg \u0026times; 2 days) failed to improve platelet counts, but oral prednisone (2 mg/kg/day) achieved complete hematologic remission by day 24 (platelets 581 \u0026times; 10\u0026sup3;/\u0026micro;L, hemoglobin 11.9 g/dL). Iron supplementation corrected the deficiency. At 20-month follow-up, sustained hematologic remission was maintained (hemoglobin 13.8 g/dL, platelets 174 \u0026times; 10\u0026sup3;/\u0026micro;L), though elevated TSH (7 \u0026micro;IU/mL) indicated steroid-related endocrine effects.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e\u003cp\u003eEvans syndrome can be masked by concurrent iron deficiency anemia in pediatric patients, emphasizing the need for comprehensive autoimmune evaluation including DAT testing in children presenting with cytopenias. Corticosteroids remain effective first-line therapy, but long-term monitoring for steroid-related complications is essential.\u003c/p\u003e","manuscriptTitle":"Unmasking the Iron Veil: A Pediatric Case Report of Evans Syndrome Complicated by Iron Deficiency Anemia","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-09-09 09:40:09","doi":"10.21203/rs.3.rs-7161064/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"reviewerAgreed","content":"14991624180457923512196084660103812068","date":"2025-09-21T12:55:06+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-30T13:45:18+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"210522465239342325632111076798145581024","date":"2025-08-30T13:01:29+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-29T14:40:50+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"136000084326985455452941716421936261137","date":"2025-08-29T14:28:51+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-08-29T12:41:58+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2025-08-04T10:17:52+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-08-01T10:13:22+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-08-01T10:09:29+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Pediatrics","date":"2025-07-19T00:51:36+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-pediatrics","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bped","sideBox":"Learn more about [BMC Pediatrics](http://bmcpediatr.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bped/default.aspx","title":"BMC Pediatrics","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"503593ba-aef6-4cd7-9107-b61006ea110c","owner":[],"postedDate":"September 9th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"under-review","subjectAreas":[],"tags":[],"updatedAt":"2025-09-09T09:40:09+00:00","versionOfRecord":[],"versionCreatedAt":"2025-09-09 09:40:09","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7161064","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7161064","identity":"rs-7161064","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}