[Danazol-induced peliosis hepatis accompanied by disseminated intravascular coagulation in a patient with myelodysplastic syndrome transformed from aplastic anemia]

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This case report describes a patient with myelodysplastic syndrome who developed peliosis hepatis and disseminated intravascular coagulation while undergoing treatment for aplastic anemia. The researchers identified the condition through imaging studies prompted by liver dysfunction, noting that the patient had been receiving danazol, an androgenic steroid hormone. Upon discontinuation of the danazol therapy, the patient’s liver injury, coagulopathy, and radiological findings showed significant improvement, suggesting a causal link between the medication and the hepatic lesions. This paper is centrally about endometriosis — specifically laparoscopic excision of deep infiltrating lesions.

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Abstract

Peliosis hepatis (PH) is a condition involving benign tumors pathologically characterized by multiple blood-filled cavities, mostly affecting the liver and spleen. Androgenic-steroids are widely used in patients with bone marrow failure syndromes (e.g.: aplastic anemia) and these patients are at increased risk of developing PH. Although patients with PH are generally asymptomatic, PH can progress to liver failure and even fatal spontaneous intraabdominal hemorrhage. Therefore, early diagnosis is critical in order to prevent life-threatening complications of PH. We herein report a patient with PH which had been treated with danazol, who presented with liver dysfunction and multiple hepatic lesions on imaging studies at the time of diagnosis. Although the patient presented with disseminated intravascular coagulation (DIC), a bone marrow biopsy revealed no evidence of leukemic transformation. The patient was diagnosed as having danazol-induced PH, and these abnormalities spontaneously resolved after the discontinuation of danazol. PH is one of the most important complications of long-term administration of androgenic-steroids. Although the mechanisms remain unclear, the multiple blood-filled cavities characteristic of PH may be responsible for the development of DIC. Therefore, monitoring of coagulation markers might also be a key strategy for early diagnosis of PH.
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症例報告 再生不良性貧血から骨髄異形成症候群に移行しDICを発症したdanazol誘発性肝ペリオーシス 2016 年 57 巻 8 号 p. 1026-1031 詳細 抄録 肝ペリオーシス(peliosis hepatis, PH)は主に肝臓や脾臓に発生する易出血性の腫瘤性病変である。血液領域では蛋白同化ホルモンを用いる再生不良性貧血などの疾患に稀に合併する。通常無症候性であるが,進行性の肝不全や肝破裂などの重篤な症状で診断された例も多く早期診断が重要である。我々はdanazolで治療中の再生不良性貧血において,肝障害に対する画像検査を契機にPHと診断した症例を経験した。DICも合併していたが,骨髄異形成症候群への移行は認めたものの,骨髄芽球は少数であった。danazol誘発性PHを疑い投与中止したところ,肝障害・DIC・画像所見は改善した。PHは蛋白同化ホルモン長期投与合併症として重要である。詳細な機序は不明であるが,多発する血液貯留腔の存在により稀にPHにDICが合併すると推測され,早期診断のため,凝固系マーカーのフォローも重要である可能性が示唆された。 © 2016 一般社団法人 日本血液学会

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MeSH descriptors

Anemia, Aplastic Bone Marrow Diseases Danazol Disseminated Intravascular Coagulation Hemoglobinuria, Paroxysmal Peliosis Hepatis Aged, 80 and over Anemia, Aplastic Bone Marrow Diseases Bone Marrow Failure Disorders Danazol Disseminated Intravascular Coagulation Female Hemoglobinuria, Paroxysmal Humans Peliosis Hepatis Treatment Outcome

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chemicals 6
danazol steroid danazol danazol danazol steroid

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