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1例甲型血友病合并严重血小板减少患者的部分脾栓塞:病例报告

Partial Splenic Embolization in a Patient with Hemophilia A and Severe Thrombocytopenia: A Case Report.

作者信息

Nakamura Tomofumi, Uchiba Mitsuhiro, Nakata Hirotomo, Mizumoto Takao, Beppu Toru, Matsushita Shuzo

机构信息

Department of Hematology, Rheumatology, and Infectious Diseases, Graduate School of Medical Sciences, Faculty of Life Sciences, Kumamoto University, Kumamoto 860-8556, Japan.

Department of Laboratory Medicine, Kumamoto University Hospital, Kumamoto 860-8556, Japan.

出版信息

Hematol Rep. 2024 Mar 26;16(2):185-192. doi: 10.3390/hematolrep16020019.

Abstract

We report a patient with hemophilia A who underwent partial splenic embolization (PSE) for severe thrombocytopenia secondary to portal hypertension-induced splenomegaly, resulting in a stable long-term quality of life. The patient was diagnosed with hemophilia A and unfortunately contracted human immunodeficiency virus (HIV), hepatitis B virus (HBV), and hepatitis C virus (HCV) from blood products. He subsequently developed progressive splenomegaly due to portal hypertension from chronic HCV, resulting in severe thrombocytopenia. PSE was performed because he had occasional subcutaneous bleeding and needed to start interferon (IFN) and ribavirin (RBV) treatment for curing his HCV infection at that time. His platelet counts increased, and no serious adverse events were observed. Currently, he continues to receive outpatient treatment, regular factor VIII (FVIII) replacement therapy for hemophilia A, and antiretroviral therapy for HIV infection. Vascular embolization has been reported to be an effective and minimally invasive treatment for bleeding in hemophilia patients. PSE also provided him with a stable quality of life without the side effects of serious infections and thrombocytopenia relapses. We conclude that PSE is a promising therapeutic option for patients with hemophilia A.

摘要

我们报告了一名甲型血友病患者,该患者因门静脉高压引起脾肿大继发严重血小板减少症而接受了部分脾栓塞术(PSE),从而获得了稳定的长期生活质量。该患者被诊断为甲型血友病,不幸的是因输血感染了人类免疫缺陷病毒(HIV)、乙型肝炎病毒(HBV)和丙型肝炎病毒(HCV)。随后,由于慢性丙型肝炎导致门静脉高压,他逐渐出现脾肿大,进而导致严重血小板减少症。当时,由于他偶尔出现皮下出血,且需要开始使用干扰素(IFN)和利巴韦林(RBV)治疗丙型肝炎感染,因此进行了PSE。他的血小板计数增加,且未观察到严重不良事件。目前,他继续接受门诊治疗,定期接受甲型血友病的凝血因子VIII(FVIII)替代治疗以及针对HIV感染的抗逆转录病毒治疗。据报道,血管栓塞术是治疗血友病患者出血的一种有效且微创的治疗方法。PSE还为他提供了稳定的生活质量,且没有严重感染和血小板减少症复发的副作用。我们得出结论,PSE对于甲型血友病患者是一种有前景的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f326/11036256/22eeb8c28b31/hematolrep-16-00019-g001.jpg

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