Effective treatment of double plasma molecular adsorption system combined with half-volume plasma exchange in Primary Systemic Light Chain Amyloidosis with hepatic involvement : a case report and review of literature

Effective treatment of double plasma molecular adsorption system combined with half-volume plasma exchange in Primary Systemic Light Chain Amyloidosis with hepatic involvement : a case report and review of literature | 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 Systematic Review Effective treatment of double plasma molecular adsorption system combined with half-volume plasma exchange in Primary Systemic Light Chain Amyloidosis with hepatic involvement : a case report and review of literature Hairong Shen, Zhiping Lv This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8644755/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Currently, the treatment of Primary Systemic Light Chain Amyloidosis (AL amyloidosis ) has improved the prognosis of patients with AL amyloidosis for the development of novel therapeutic options and a better understanding of the supportive care.However,the morbidity and mortality rates of AL amyloidosis still remain high.So it is very important and imperative to find new ways and drugs for the treatment of AL amyloidosis. Here we report an unusual case of AL amyloidosis with hepatic involvement for the effective treatment in the severe hyperbilirubinemia using the double plasma molecular adsorption system (DPMAS) combined with plasma exchange therapy. hepatic involvement of AL amyloidosis double plasma molecular adsorption system (DPMAS) combined with plasma exchange Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Introduction Primary systemic light chain amyloidosis(AL amyloidosis) can present with varied and unusual initial symptoms, making diagnosis difficult without high clinical suspicion[ 1 ]. Due to the lack of specific symptoms,the duration between the onset of symptoms and the diagnosis of AL amyloidosis is usually long[ 2 ]. The duration in previous reports was 6–10 months [ 3 – 5 ]. One study, however, reported that only 7.6% of patients received a diagnosis of amyloidosis after visiting one physician, and that 31.8% visited more than 5 physicians before receiving the correct diagnosis [ 6 ].Hepatic involvement in AL amyloidosis is very common but remains a challenge for doctors because of its typically presentations with nonspecific symptoms.Even though the new and current treatment Daratumumab, a human CD38-targeting antibody, has shown efficacy in improving hematological parameters and organ function in patients with AL amyloidosis[ 7 ],but not every patient with AL amyloidosis is suitable to have Daratumumab ,nor can every patient in developing countries afford the cost of Daratumumab. So it is very important and imperative to find new ways and drugs for the treatment of AL amyloidosis.To our knowledge,cases of AL amyloidosis involving the liver being treated with DPMAS combined with plasma exchange in hyperbilirubin have not been reported previously. Here we report an unusual case of AL amyloidosis with hepatic involvement for the effective treatment in the severe hyperbilirubinemia using the double plasma molecular adsorption system (DPMAS) combined with plasma exchange therapy. Case Presentation We describe a case of 58-year-old gentleman presenting with abdominal distension associated with jaundice, pain in knees, ankles and the first toe of both feet.Occasionally, chest tightness and discomfort were obvious after activities, lasting 3–5 minutes each time, and could be relieved by themselves after rest. Occasionally, there was a dull abdominal pain.The patient had histories of hypertension,gouty arthritis,thalassemia,iron deficiency anemia,chronic atrophic gastritis,rectal poyp, lumbar trauma and lumbar plate implantation.No history of parasite,alcohol,illicit drugs, herbal products, or hepatotoxic medication use. No high risk sexual behavior. No family history of liver diseases,genetic or tumor diseases.On physical examination,he had mild jaundice of skin and sclera.And a movable,non-tender, non-ulcerated mass approximately 2*2cm in size was palpable in his left neck.The lower border of the liver could be palpable without tenderness and percussion pain in his right lower abdomen, about 3cm from the costal margin. On evaluation he had raised bilirubin,alkaline phosphatase and raised GGT levels with lightly elevated transaminases.His viral markers were negative.His autoimmune markers were negative except for ANA. Four items of liver fibrosis were: serum hyaluronidase level 161.00 ng/ml, serum type III collagen level 290.00 ng/ml, serum laminin level 146.00 ng/ml, serum type IV collagen level 89.50 ng/ml.An abnormal spike was seen in the γ region of serum protein electrophoresis, which was suspected to be M protein. Immunofixation electrophoresis was recommended.Serum immunofixation electrophoresis(IFE)revealed monoclonal IgM andκlight chain(Fig. 1 ); The outcome of bone marrow aspiration demonstrated plasma cell account for 6.5%, and part of lymphocytes demonstrate polymorphic changes. The bone marrow conclusion directed to lymphomaplasmacytic lymphoma or Waldenström macroglobulinemia(LPL/WM)(Fig. 2 ).On imaging he had liver steatosis(fatty liver); multiple nodular and significantly enhanced lesions in the spleen, consider hemangioma; gallbladder stones.Routine ultrasound examination of body surface mass demonstrated subcutaneous hypoechoic areas were seen in both neck regions (partial enlargement of lymph nodes?).His hepatic stiffness score of Fibro Scan was 41Kpa. Other biochemical indicators and auxiliary examinations of this patient were normal or showed no significant abnormalities. After his admission, we gave this patient conventional liver-protected drugs for several days.However,the patient’s liver function had no sign of improvement ,his bilirubin hadn’t been decreased but increased(with the total bililrubin from 102.1 to 142.8 umol/L). We realized we might run into a kind of liver disease which is difficult to diagnose. So we turned to the liver biopsy. During the liver biopsy, we fetched two samples, one for our hospital pathology department,one for Guangxi Kingmed center for clinical laboratory,with the purpose of preventing misdiagnosis or delay of the diagnosis.The Guangxi Kingmed center for clinical laboratory offers consultation and service for difficult and complex liver diseases.Our pathology department was first to send the outcome of liver biopsy to us but failed to give any useful clues for the diagnosis.Fortunately,the pathology report of Guangxi Kingmed center for clinical laboratory gave us the right direction for the diagnosis. The detailed description of the report and pathology images(Fig. 3 ) were as below:1)There is more deposition of light pink matter in hepatocyte space and perisinusoidal space, hepatocyte atrophy, narrowing or occlusion of hepatic sinuses, infiltration of lymphocytes and a few neutrophils in the portal area, and deposition of light pink matter can be seen in the stroma. 2)Immunohistochemistry: CK7, CK19 bile duct epithelium +, mild bile duct reaction, CK7 positive hepatocytes accounted for about 30%; CD68 showed proliferation and activation of Kupffer cells; α- SMA slightly activated hepatic stellate cells; MUM1 + plasma cells; IgG-, IgG4-。3)Special staining: Deposits in hepatocyte space and perisinusoidal space, Masson staining was light blue; Sirius scarlet staining is red; Reticular scaffolds were found in the hepatic plate by reticular staining; PAS hepatocytes +, d-pas -, Prussian blue -, copper staining -.Their original result:Consider hepatic amyloidosis;Immunohistochemical staining is recommended: AA, κ, λ, CD138, IgM, IgA, and special stains: Congo red, ox-Congo red for collaborating diagnosis and typing. Because of the recommendation of the pathology report from Guangxi Kingmed center for clinical laboratory,the patient underwent bone marrow biopsy, bone marrow cell flow cytometry, and left cervical lymph node biopsy. The bone marrow biopsy prestented that there is a lot of amyloid in the interstitium, starch staining (+);and flow cytometry immunophemtyping of bone marrow cells revealed: About 10.1% mature B lymphocytes were seen,their immunophenotypes were CD19+,CD20+, CD5-, CD10-, CD103-,CD11c-,CD123-,CD25 + part,CD22+,CD200 + part,and membrane immunoglobulin Kappa light Chain restricted expression(Fig. 4 ). Our pathology department conducted Congo red staining for the patient’s liver ,bone marrow and left cervical lymph node. All the Congo red stainings above were positive:apple-green birefringence were all seen under polarized light(Fig. 5 ).Finally, the diagnosis of primary systemic light chain amyloidosis and primary hepatic amyloidosis for this patient had been confirmed. About10.1% mature B lymphocytes were seen, their immunophenotypes were CD19+,CD20+,CD5-,CD10-,CD103-,CD11c-,CD123-,CD25 + part,CD22+,CD200 + part, and membrane immunoglobulin Kappa light Chain restricted expression. As it had been refered that the patient had abnormal liver function after hospitalization in 2022.And the patient used to be hospitalized in Minzu Hospital of Guangxi Medical University in 2021.Table 1 shows the dynamic changes of the patient’s liver function during the hospitalization in Minzu Hospital of Guangxi Medical University.From Table 1 ,we can see that this patient’s GGT and ALP started to increase while TBIL,DBIL and IBIL were normal in 2021,one year before he demonstrated abdominal symptoms.And after his admission into our department of gastroenterology and hematology, his bilirubin gradually increased to the peak of 356.6 umol/L, which were nearly to the edge of liver failure. The conventional treatment was ineffective at all. In order to prevent the risk of liver failure and decrease the bilirubin, we conducted two course of DPMAS combined with half-volume plasma exchange.To our surprise, the index of bilirbubin of this patient decreased apparently including the levels of GGT, ALP and TBA. Because of the efficiency of DPMAS combined with plasma exchange,our patient had a good chance to start chemotherapy. He was treated with BD(Bortezomib and Dexamethasone)plan.During the chemotherapy, his liver function maintained a stable state including bilirubin. Jaundice never appeared again after two course of DPMAS combined with plasma exchange therapy.Unfortunately, this patient gave up the treatment after several circles of chemotherapy,and he died in the end due to the progression of the AL amyloidosis after two months apart from Minzu Hospital of Guangxi Medical University. Discussion Amyloidosis is a systemic disease caused by the extracellular deposition of misfolded fibrillar proteins leading to dysfunction of the affected organ[ 8 ]. The most common subgroup is AL (amyloidosis, light chain) -amyloidosis with an incidence of 5–13 in one million personyears [ 9 ]. Hepatic involvement in systemic amyloidosis is common and occurs in myeloma-related (AL) amyloidosis (primary) and amyloid-associated (AA) amyloidosis (secondary or reactive)[ 10 ].There are many case reports about the AL amyloidosis with hepatic involvement.However,the present case here is unique for several aspects as below. First, the diagnosis of primary systemic light chain amyloidosis didn’t take us much time thanks for the first sign of abnormal serum protein electrophoresis and the pathology report of Guangxi Kingmed center for clinical laboratory. Besides,timely and correct clinical decision makings were made from the efforts and collaborations of gastroenterologists and hemotologists because the department the patient admitted is gastroenterology and hemotology. The whole procedure and course demonstrates that the multi-disciplinay team collaboration plays an important and essential role in the diagnosis and treatment of the disease. Second,the patient’s Fibro Scan outcome presented his hepatic stiffness score was 41Kpa,and four items of liver fibrosis were all high,while his abdomial CT demonstrated fatty liver. This finding is in line with Brunger’s report and backs up his conclusion that liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement[ 11 ].In addition,our result is also consistent with one study,demonstating that high score of liver stiffness measured by Fibro Scan technique may be of value in patients with known amyloidosis, especially in whom liver biopsy is unavailable[ 12 ].However,more clinical and laboratory work up are needed to be done because it is still difficult to distinguish amyloidosis from other cirrhotic liver disease . It was reported that the mean survival time of the patients with AL amyloidosis ranges between 12 and 18 months[ 13 , 14 ],the hepatic involvement[ 15 ]decreases the survival time to between 10 and 14 months, the presence of cholestatic jaundice to 3–5 months[ 15 , 16 ]. But our patient survived to nearly a year after diagnosis even though he had cholestatic jaundice. The difference was our patient gained timely DPMAS combined with half-volume plasma exchange(one mode of non-bioartificial liver support systems) therapy. As we all know,Plasma Exchange (PE)treatment is widely used in many countries [ 17 , 18 ], and it can improve liver function and the short-term prognosis of patients with liver failure [ 19 , 20 ]. Plasma adsorption perfusion is an extracorporeal liver support technique in which bilirubin is removed from the plasma through a specific adsorbing cartridge. Double plasma molecular adsorption system adds a broad-spectrum adsorption column for the removal of inflammatory mediators and antibodies and other medium toxins. Their use in the treatment of hyperbilirubinemia has been established with several emerging data indicating their efficacy when compared to other extracorporeal techniques[ 21 ].Our present patient’s experience of the DPMAS combined with half-volume plasma exchange therapy also confirmed its efficacy. At the first time of his hospitalization in Minzu Hospital of Guangxi Medical University,our patient’s GGT and ALP started to increase while TBIL,DBIL and IBIL were normal in 2021(Table 1 ),one year before he demonstrated abdominal symptoms. It conformed to Vilma Takayasu’s conclusion that the clinical features of hepatic amyloidosis are generally mild. Hepatomegaly and alkaline phosphatase elevation are the most common findings[ 22 ].And after his second time of hospitalization in Minzu Hospital of Guangxi Medical University in 2022, his bilirubin gradually increased to the peak of 356.6 umol/L, which were nearly to the edge of liver failure. In order to prevent the risk of liver failure and decrease the bilirubin, we conducted two course of DPMAS combined with half-volume plasma exchange.To our surprise, the index of bilirbubin of this patient decreased apparently including the levels of GGT, ALP and TBA.This patient’s jaundice gradually relapse after taking two course of DPMAS combined with plasma exchange therapy.and our patient survived to nearly a year after diagnosis even though he had cholestatic jaundice. This discovery subverted past knowledge because previous studies demonstrated that cholestatic jaundice, is associated with poor prognosis in AL amyloidosis patients with the involvement of liver and with whose survival time to 3–5 months[ 16 ]. Wu et al.used to report that significant decline was noticed in the TBIL, direct bilirubin (DBIL), total bile acid after DPMAS combined with plasma exchange therapy on the acute severe Cholestatic Hepatitis[ 23 ].Besides,Liu et al.reported that DPMAS combined with plasma exchange therapy is effective in controlling thyroid storm with severe liver injury[ 24 ].Now we find that DPMAS therapy also can be applicable to AL amyloidosis patients with cholestatic juandiice whose livers were involvement.It gives us a good way to relapse jaundice in AL amyloidosis patients.We guess it might be the first time for DPMAS combined with plasma exchange being used in the treatment of jaundice in primary systemic light chain amyloidosis with hepatic involvement.To our knowledge,cases of AL amyloidosis involving the liver being treated with DPMAS combined with plasma exchange in hyperbilirubinemia have not been reported previously. Conclusion The present case indicates that DPMAS combined with plasma exchange treatment can have efficacy in controlling severe hyperbilirubinemia in AL amyloidosis with hepetic involvement.However,this is just a case report, further studies involving more patients and application data of DPMAS combined with plasma exchange in the treatment of hyperbilirubinemia of AL amyloidosis with hepatic involvement are needed to confirm the efficacy of DPMAS combined with plasma exchange therapy. Declarations the patient's family consented to participate and publish their clinical case. Conflict of Interest The authors declare that they have no conflict of interest. Author Contribution Hairong Shen wrote the manuscript and prepared figure 1-5.All authors reviewed the manuscript. Acknowledgement We thank medical laboratory technician Zhifeng Wei from Department of Pathology of RuiKang Hospital Affiliated to Guangxi University of Chinese Medicine,Nanning,China for his technical assistance in polarized light microphotography. References Haley EM, Nabatian AS, Kopp SA, Falasca GF, Haupt HM, Halpern AV. Systemic amyloidosis: unusual presentation mistaken for a recurrent scabies infection. Cutis. 2014;93(6):311-5. PMID: 24999644. Karasuyama T, Honma Y, Kumamoto K, Shibata M, Watanabe T, Shimajiri S, Abe S, Yamashita T, Harada M. Hepatocyte Growth Factor and Primary Systemic Amyloidosis. J UOEH. 2021;43(2):227–233. 10.7888/juoeh.43.227 . PMID: 34092767. Palladini G, Kyle RA, Larson DR, Therneau TM, Merlini G, Gertz MA. Multicentre versus single centre approach to rare diseases: the model of systemic light chain amyloidosis. Amyloid. 2005;12(2):120–6. Huang XH, Liu ZH. The Clinical Presentation and Management of Systemic Light-Chain Amyloidosis in China. 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Guidelines on the use of therapeutic apheresis in clinical practice - evidence-based approach from the writing committee of the American society for apheresis: the ninth special issue. J Clin Apher. 2023;38(2):77–278. Chen Y, Han T, Duan Z. Clinical application of artificial liver and blood purification: expert consensus recommendations. Hepatol Int. 2023;17(1):4–17. Larsen FS, Schmidt LE, Bernsmeier C, Rasmussen A, Isoniemi H, Patel VC, et al. High-volume plasma exchange in patients with acute liver failure: an open randomised controlled trial. J Hepatol. 2016;64(1):69–78. Maiwall R, Bajpai M, Singh A, Agarwal T, Kumar G, Bharadwaj A, et al. Standard-volume plasma exchange improves outcomes in patients with acute liver failure: A randomized controlled trial. Clin Gastroenterol Hepatol. 2022;20(4):e831–54. Marcello M, Ronco C. Bilirubin Adsorption with DPMAS: Mechanism of Action and Efficacy of Anion Exchange Resin. Contrib Nephrol. 2023;200:201–9. 10.1159/000526729 . Epub 2023 Jun 1. 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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-8644755","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Systematic Review","associatedPublications":[],"authors":[{"id":577244467,"identity":"fbe49b26-6893-4ee9-b075-031b40375103","order_by":0,"name":"Hairong Shen","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAzUlEQVRIiWNgGAWjYBACxmb+BwYf/7HJsbE3EKmFuZ2HoXAGG58xP88BIrWw9/MwfOZhk0uUnJFApBbeZt6DG2fwmCUY3Hy88QZDjU00QS2SzXzJBh8k0vIMbqcVWzAcS8ttIKTFsJnBzHCGwbFig9s5ZhKMDYcJa7E/zGD+myfhf+KGm2eI1MLYzGNgzHOALXEm0EPEamFLMJzZwAYMZKBfEojxC2P/4QMGHxtAUXl4440PNTaEtSADA4kEUpRDtJCqYxSMglEwCkYGAAC2Uz7ru4gkbwAAAABJRU5ErkJggg==","orcid":"","institution":"Shenzhen Pingle Orthopedic Hospital","correspondingAuthor":true,"prefix":"","firstName":"Hairong","middleName":"","lastName":"Shen","suffix":""},{"id":577244468,"identity":"8f709e44-6c49-4ce4-892f-1d65ced64acf","order_by":1,"name":"Zhiping Lv","email":"","orcid":"","institution":"Southern Medical University of Integrated Traditional Chinese and Western Medicine","correspondingAuthor":false,"prefix":"","firstName":"Zhiping","middleName":"","lastName":"Lv","suffix":""}],"badges":[],"createdAt":"2026-01-20 04:23:51","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-8644755/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-8644755/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":101935755,"identity":"fe10d54f-f7da-4248-8627-9712a85ae260","added_by":"auto","created_at":"2026-02-05 08:28:22","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":14266,"visible":true,"origin":"","legend":"\u003cp\u003eSerum immunofixation electrophoresis (IFE) revealed monoclonal IgM andκlight chain.\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-8644755/v1/ce9f5e6f10d8b29b3c2c0610.png"},{"id":101935667,"identity":"45c37a02-244d-44a1-88b7-f946cb23544f","added_by":"auto","created_at":"2026-02-05 08:28:04","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":41389,"visible":true,"origin":"","legend":"\u003cp\u003ebone marrow aspiration demonstrated plasma cell account for 6.5%, and part of lymphocytes demonstrate polymorphic changes\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-8644755/v1/66dd9fe2f3378ff43fadfe84.png"},{"id":101935649,"identity":"17da96c6-8745-4c8a-b763-4090a925ea74","added_by":"auto","created_at":"2026-02-05 08:27:51","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":306504,"visible":true,"origin":"","legend":"\u003cp\u003ethe pathology image and description of Guangxi Kingmed center for clinical laboratoy:a)Deposition of light pink matter in hepatocyte space and perisinusoidal space;b)CK7 ;c) MUM1; d)Masson staining was light blue;e)Sirian red staining is red\u003c/p\u003e","description":"","filename":"floatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-8644755/v1/f7760afc3366ff6df8171ad7.png"},{"id":101935658,"identity":"903d958e-3339-4006-9ee6-8a6993d82536","added_by":"auto","created_at":"2026-02-05 08:27:54","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":185773,"visible":true,"origin":"","legend":"\u003cp\u003eflow cytometry immunophemtyping of bone marrow cells:\u003c/p\u003e\n\u003cp\u003eAbout10.1% mature B lymphocytes were seen, their immunophenotypes were CD19+,CD20+,CD5-,CD10-,CD103-,CD11c-,CD123-,CD25+part,CD22+,CD200+part, and membrane immunoglobulin Kappa light Chain restricted expression.\u003c/p\u003e","description":"","filename":"floatimage4.png","url":"https://assets-eu.researchsquare.com/files/rs-8644755/v1/d684029cd3589d39626ac2a1.png"},{"id":101935690,"identity":"24545031-66aa-496c-9a92-29dbe2d082fe","added_by":"auto","created_at":"2026-02-05 08:28:15","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":6185588,"visible":true,"origin":"","legend":"\u003cp\u003eMicroscopic observations:A,C,E were H\u0026amp;E staining,200x; B,D,F were Congo Red stainings, showing apple-green birefringence in polarized light,200x\u003c/p\u003e","description":"","filename":"floatimage5.png","url":"https://assets-eu.researchsquare.com/files/rs-8644755/v1/a2893d43e6d0ef5f6f2a9fa7.png"},{"id":102236011,"identity":"0bcc4309-c321-48f0-b1c0-c908e1a23ddb","added_by":"auto","created_at":"2026-02-09 16:18:35","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":7120118,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8644755/v1/c1225b24-0da3-405d-905f-a890aa3858c0.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Effective treatment of double plasma molecular adsorption system combined with half-volume plasma exchange in Primary Systemic Light Chain Amyloidosis with hepatic involvement : a case report and review of literature","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePrimary systemic light chain amyloidosis(AL amyloidosis) can present with varied and unusual initial symptoms, making diagnosis difficult without high clinical suspicion[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Due to the lack of specific symptoms,the duration between the onset of symptoms and the diagnosis of AL amyloidosis is usually long[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. The duration in previous reports was 6\u0026ndash;10 months [\u003cspan additionalcitationids=\"CR4\" citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. One study, however, reported that only 7.6% of patients received a diagnosis of amyloidosis after visiting one physician, and that 31.8% visited more than 5 physicians before receiving the correct diagnosis [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].Hepatic involvement in AL amyloidosis is very common but remains a challenge for doctors because of its typically presentations with nonspecific symptoms.Even though the new and current treatment Daratumumab, a human CD38-targeting antibody, has shown efficacy in improving hematological parameters and organ function in patients with AL amyloidosis[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e],but not every patient with AL amyloidosis is suitable to have Daratumumab ,nor can every patient in developing countries afford the cost of Daratumumab. So it is very important and imperative to find new ways and drugs for the treatment of AL amyloidosis.To our knowledge,cases of AL amyloidosis involving the liver being treated with DPMAS combined with plasma exchange in hyperbilirubin have not been reported previously. Here we report an unusual case of AL amyloidosis with hepatic involvement for the effective treatment in the severe hyperbilirubinemia using the double plasma molecular adsorption system (DPMAS) combined with plasma exchange therapy.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eWe describe a case of 58-year-old gentleman presenting with abdominal distension associated with jaundice, pain in knees, ankles and the first toe of both feet.Occasionally, chest tightness and discomfort were obvious after activities, lasting 3\u0026ndash;5 minutes each time, and could be relieved by themselves after rest. Occasionally, there was a dull abdominal pain.The patient had histories of hypertension,gouty arthritis,thalassemia,iron deficiency anemia,chronic atrophic gastritis,rectal poyp, lumbar trauma and lumbar plate implantation.No history of parasite,alcohol,illicit drugs, herbal products, or hepatotoxic medication use. No high risk sexual behavior. No family history of liver diseases,genetic or tumor diseases.On physical examination,he had mild jaundice of skin and sclera.And a movable,non-tender, non-ulcerated mass approximately 2*2cm in size was palpable in his left neck.The lower border of the liver could be palpable without tenderness and percussion pain in his right lower abdomen, about 3cm from the costal margin.\u003c/p\u003e\n\u003cp\u003eOn evaluation he had raised bilirubin,alkaline phosphatase and raised GGT levels with lightly elevated transaminases.His viral markers were negative.His autoimmune markers were negative except for ANA. Four items of liver fibrosis were: serum hyaluronidase level 161.00 ng/ml, serum type III collagen level 290.00 ng/ml, serum laminin level 146.00 ng/ml, serum type IV collagen level 89.50 ng/ml.An abnormal spike was seen in the \u0026gamma; region of serum protein electrophoresis, which was suspected to be M protein. Immunofixation electrophoresis was recommended.Serum immunofixation electrophoresis(IFE)revealed monoclonal IgM and\u0026kappa;light chain(Fig.\u0026nbsp;\u003cspan\u003e1\u003c/span\u003e); The outcome of bone marrow aspiration demonstrated plasma cell account for 6.5%, and part of lymphocytes demonstrate polymorphic changes. The bone marrow conclusion directed to lymphomaplasmacytic lymphoma or Waldenstr\u0026ouml;m macroglobulinemia(LPL/WM)(Fig.\u0026nbsp;\u003cspan\u003e2\u003c/span\u003e).On imaging he had liver steatosis(fatty liver); multiple nodular and significantly enhanced lesions in the spleen, consider hemangioma; gallbladder stones.Routine ultrasound examination of body surface mass demonstrated subcutaneous hypoechoic areas were seen in both neck regions (partial enlargement of lymph nodes?).His hepatic stiffness score of Fibro Scan was 41Kpa. Other biochemical indicators and auxiliary examinations of this patient were normal or showed no significant abnormalities.\u003c/p\u003e\n\u003cp\u003eAfter his admission, we gave this patient conventional liver-protected drugs for several days.However,the patient\u0026rsquo;s liver function had no sign of improvement ,his bilirubin hadn\u0026rsquo;t been decreased but increased(with the total bililrubin from 102.1 to 142.8 umol/L). We realized we might run into a kind of liver disease which is difficult to diagnose. So we turned to the liver biopsy. During the liver biopsy, we fetched two samples, one for our hospital pathology department,one for Guangxi Kingmed center for clinical laboratory,with the purpose of preventing misdiagnosis or delay of the diagnosis.The Guangxi Kingmed center for clinical laboratory offers consultation and service for difficult and complex liver diseases.Our pathology department was first to send the outcome of liver biopsy to us but failed to give any useful clues for the diagnosis.Fortunately,the pathology report of Guangxi Kingmed center for clinical laboratory gave us the right direction for the diagnosis. The detailed description of the report and pathology images(Fig.\u0026nbsp;\u003cspan\u003e3\u003c/span\u003e) were as below:1)There is more deposition of light pink matter in hepatocyte space and perisinusoidal space, hepatocyte atrophy, narrowing or occlusion of hepatic sinuses, infiltration of lymphocytes and a few neutrophils in the portal area, and deposition of light pink matter can be seen in the stroma. 2)Immunohistochemistry: CK7, CK19 bile duct epithelium +, mild bile duct reaction, CK7 positive hepatocytes accounted for about 30%; CD68 showed proliferation and activation of Kupffer cells; \u0026alpha;- SMA slightly activated hepatic stellate cells; MUM1\u0026thinsp;+\u0026thinsp;plasma cells; IgG-, IgG4-。3)Special staining: Deposits in hepatocyte space and perisinusoidal space, Masson staining was light blue; Sirius scarlet staining is red; Reticular scaffolds were found in the hepatic plate by reticular staining; PAS hepatocytes +, d-pas -, Prussian blue -, copper staining -.Their original result:Consider hepatic amyloidosis;Immunohistochemical staining is recommended: AA, \u0026kappa;, \u0026lambda;, CD138, IgM, IgA, and special stains: Congo red, ox-Congo red for collaborating diagnosis and typing.\u003c/p\u003e\n\u003cp\u003eBecause of the recommendation of the pathology report from Guangxi Kingmed center for clinical laboratory,the patient underwent bone marrow biopsy, bone marrow cell flow cytometry, and left cervical lymph node biopsy. The bone marrow biopsy prestented that there is a lot of amyloid in the interstitium, starch staining (+);and flow cytometry immunophemtyping of bone marrow cells revealed: About 10.1% mature B lymphocytes were seen,their immunophenotypes were CD19+,CD20+, CD5-, CD10-, CD103-,CD11c-,CD123-,CD25\u0026thinsp;+\u0026thinsp;part,CD22+,CD200\u0026thinsp;+\u0026thinsp;part,and membrane immunoglobulin Kappa light Chain restricted expression(Fig. \u003cspan\u003e4\u003c/span\u003e). Our pathology department conducted Congo red staining for the patient\u0026rsquo;s liver ,bone marrow and left cervical lymph node. All the Congo red stainings above were positive:apple-green birefringence were all seen under polarized light(Fig. \u003cspan\u003e5\u003c/span\u003e).Finally, the diagnosis of primary systemic light chain amyloidosis and primary hepatic amyloidosis for this patient had been confirmed.\u003c/p\u003e\n\u003cp\u003eAbout10.1% mature B lymphocytes were seen, their immunophenotypes were CD19+,CD20+,CD5-,CD10-,CD103-,CD11c-,CD123-,CD25\u0026thinsp;+\u0026thinsp;part,CD22+,CD200\u0026thinsp;+\u0026thinsp;part, and membrane immunoglobulin Kappa light Chain restricted expression.\u003c/p\u003e\n\u003cdiv\u003e\n \u003cdiv align=\"left\"\u003e\u003cimg src=\"https://myfiles.space/user_files/122228_c8a1650c59388082/122228_custom_files/img1770272691.png\"\u003e\u003c/div\u003e\n\u003c/div\u003e\n\u003cp\u003eAs it had been refered that the patient had abnormal liver function after hospitalization in 2022.And the patient used to be hospitalized in Minzu Hospital of Guangxi Medical University in 2021.Table\u0026nbsp;\u003cspan\u003e1\u003c/span\u003e shows the dynamic changes of the patient\u0026rsquo;s liver function during the hospitalization in Minzu Hospital of Guangxi Medical University.From Table \u003cspan\u003e1\u003c/span\u003e,we can see that this patient\u0026rsquo;s GGT and ALP started to increase while TBIL,DBIL and IBIL were normal in 2021,one year before he demonstrated abdominal symptoms.And after his admission into our department of gastroenterology and hematology, his bilirubin gradually increased to the peak of 356.6 umol/L, which were nearly to the edge of liver failure. The conventional treatment was ineffective at all. In order to prevent the risk of liver failure and decrease the bilirubin, we conducted two course of DPMAS combined with half-volume plasma exchange.To our surprise, the index of bilirbubin of this patient decreased apparently including the levels of GGT, ALP and TBA. Because of the efficiency of DPMAS combined with plasma exchange,our patient had a good chance to start chemotherapy. He was treated with BD(Bortezomib and Dexamethasone)plan.During the chemotherapy, his liver function maintained a stable state including bilirubin. Jaundice never appeared again after two course of DPMAS combined with plasma exchange therapy.Unfortunately, this patient gave up the treatment after several circles of chemotherapy,and he died in the end due to the progression of the AL amyloidosis after two months apart from Minzu Hospital of Guangxi Medical University.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eAmyloidosis is a systemic disease caused by the extracellular deposition of misfolded fibrillar proteins leading to dysfunction of the affected organ[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. The most common subgroup is AL (amyloidosis, light chain) -amyloidosis with an incidence of 5\u0026ndash;13 in one million personyears [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Hepatic involvement in systemic amyloidosis is common and occurs in myeloma-related (AL) amyloidosis (primary) and amyloid-associated (AA) amyloidosis (secondary or reactive)[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e].There are many case reports about the AL amyloidosis with hepatic involvement.However,the present case here is unique for several aspects as below.\u003c/p\u003e \u003cp\u003eFirst, the diagnosis of primary systemic light chain amyloidosis didn\u0026rsquo;t take us much time thanks for the first sign of abnormal serum protein electrophoresis and the pathology report of Guangxi Kingmed center for clinical laboratory. Besides,timely and correct clinical decision makings were made from the efforts and collaborations of gastroenterologists and hemotologists because the department the patient admitted is gastroenterology and hemotology. The whole procedure and course demonstrates that the multi-disciplinay team collaboration plays an important and essential role in the diagnosis and treatment of the disease.\u003c/p\u003e \u003cp\u003eSecond,the patient\u0026rsquo;s Fibro Scan outcome presented his hepatic stiffness score was 41Kpa,and four items of liver fibrosis were all high,while his abdomial CT demonstrated fatty liver. This finding is in line with Brunger\u0026rsquo;s report and backs up his conclusion that liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement[\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e].In addition,our result is also consistent with one study,demonstating that high score of liver stiffness measured by Fibro Scan technique may be of value in patients with known amyloidosis, especially in whom liver biopsy is unavailable[\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e].However,more clinical and laboratory work up are needed to be done because it is still difficult to distinguish amyloidosis from other cirrhotic liver disease .\u003c/p\u003e \u003cp\u003eIt was reported that the mean survival time of the patients with AL amyloidosis ranges between 12 and 18 months[\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e],the hepatic involvement[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]decreases the survival time to between 10 and 14 months, the presence of cholestatic jaundice to 3\u0026ndash;5 months[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. But our patient survived to nearly a year after diagnosis even though he had cholestatic jaundice. The difference was our patient gained timely DPMAS combined with half-volume plasma exchange(one mode of non-bioartificial liver support systems) therapy.\u003c/p\u003e \u003cp\u003eAs we all know,Plasma Exchange (PE)treatment is widely used in many countries [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e, \u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e], and it can improve liver function and the short-term prognosis of patients with liver failure [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e, \u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]. Plasma adsorption perfusion is an extracorporeal liver support technique in which bilirubin is removed from the plasma through a specific adsorbing cartridge. Double plasma molecular adsorption system adds a broad-spectrum adsorption column for the removal of inflammatory mediators and antibodies and other medium toxins. Their use in the treatment of hyperbilirubinemia has been established with several emerging data indicating their efficacy when compared to other extracorporeal techniques[\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e].Our present patient\u0026rsquo;s experience of the DPMAS combined with half-volume plasma exchange therapy also confirmed its efficacy.\u003c/p\u003e \u003cp\u003eAt the first time of his hospitalization in Minzu Hospital of Guangxi Medical University,our patient\u0026rsquo;s GGT and ALP started to increase while TBIL,DBIL and IBIL were normal in 2021(Table \u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e),one year before he demonstrated abdominal symptoms. It conformed to Vilma Takayasu\u0026rsquo;s conclusion that the clinical features of hepatic amyloidosis are generally mild. Hepatomegaly and alkaline phosphatase elevation are the most common findings[\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e].And after his second time of hospitalization in Minzu Hospital of Guangxi Medical University in 2022, his bilirubin gradually increased to the peak of 356.6 umol/L, which were nearly to the edge of liver failure. In order to prevent the risk of liver failure and decrease the bilirubin, we conducted two course of DPMAS combined with half-volume plasma exchange.To our surprise, the index of bilirbubin of this patient decreased apparently including the levels of GGT, ALP and TBA.This patient\u0026rsquo;s jaundice gradually relapse after taking two course of DPMAS combined with plasma exchange therapy.and our patient survived to nearly a year after diagnosis even though he had cholestatic jaundice. This discovery subverted past knowledge because previous studies demonstrated that cholestatic jaundice, is associated with poor prognosis in AL amyloidosis patients with the involvement of liver and with whose survival time to 3\u0026ndash;5 months[\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. Wu et al.used to report that significant decline was noticed in the TBIL, direct bilirubin (DBIL), total bile acid after DPMAS combined with plasma exchange therapy on the acute severe Cholestatic Hepatitis[\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e].Besides,Liu et al.reported that DPMAS combined with plasma exchange therapy is effective in controlling thyroid storm with severe liver injury[\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e].Now we find that DPMAS therapy also can be applicable to AL amyloidosis patients with cholestatic juandiice whose livers were involvement.It gives us a good way to relapse jaundice in AL amyloidosis patients.We guess it might be the first time for DPMAS combined with plasma exchange being used in the treatment of jaundice in primary systemic light chain amyloidosis with hepatic involvement.To our knowledge,cases of AL amyloidosis involving the liver being treated with DPMAS combined with plasma exchange in hyperbilirubinemia have not been reported previously.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThe present case indicates that DPMAS combined with plasma exchange treatment can have efficacy in controlling severe hyperbilirubinemia in AL amyloidosis with hepetic involvement.However,this is just a case report, further studies involving more patients and application data of DPMAS combined with plasma exchange in the treatment of hyperbilirubinemia of AL amyloidosis with hepatic involvement are needed to confirm the efficacy of DPMAS combined with plasma exchange therapy.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cspan\u003ethe patient\u0026apos;s family consented to participate and publish their clinical case.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e \u003ch2\u003eConflict of Interest\u003c/h2\u003e \u003cp\u003eThe authors declare that they have no conflict of interest.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eHairong Shen wrote the manuscript and prepared figure 1-5.All authors reviewed the manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgement\u003c/h2\u003e\u003cp\u003eWe thank medical laboratory technician Zhifeng Wei from Department of Pathology of RuiKang Hospital Affiliated to Guangxi University of Chinese Medicine,Nanning,China for his technical assistance in polarized light microphotography.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eHaley EM, Nabatian AS, Kopp SA, Falasca GF, Haupt HM, Halpern AV. 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PMID: 41430978; PMCID: PMC12727359.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"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":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"hepatic involvement of AL amyloidosis, double plasma molecular adsorption system (DPMAS) combined with plasma exchange","lastPublishedDoi":"10.21203/rs.3.rs-8644755/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8644755/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eCurrently, the treatment of Primary Systemic Light Chain Amyloidosis (AL amyloidosis ) has improved the prognosis of patients with AL amyloidosis for the development of novel therapeutic options and a better understanding of the supportive care.However,the morbidity and mortality rates of AL amyloidosis still remain high.So it is very important and imperative to find new ways and drugs for the treatment of AL amyloidosis. Here we report an unusual case of AL amyloidosis with hepatic involvement for the effective treatment in the severe hyperbilirubinemia using the double plasma molecular adsorption system (DPMAS) combined with plasma exchange therapy.\u003c/p\u003e","manuscriptTitle":"Effective treatment of double plasma molecular adsorption system combined with half-volume plasma exchange in Primary Systemic Light Chain Amyloidosis with hepatic involvement : a case report and review of literature","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-02-05 08:23:40","doi":"10.21203/rs.3.rs-8644755/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"a18fb607-ead5-4508-b02b-e496ef901518","owner":[],"postedDate":"February 5th, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-02-09T16:17:01+00:00","versionOfRecord":[],"versionCreatedAt":"2026-02-05 08:23:40","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8644755","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8644755","identity":"rs-8644755","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}