Acquired Factor XIII inhibitor associated with mantle cell lymphoma.
Acquired Factor XIII inhibitor associated with mantle cell lymphoma.
复制标题
与套细胞淋巴瘤相关的获得性因子 XIII 抑制剂。
DOI：
10.1111/trf.13947
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发表时间：
2017
期刊：
影响因子：
2.9
通讯作者：
Sweeney,JosephD
中科院分区：
文献类型：
--
作者：
Nixon,ChristianP;Prsic,ElizabethH;Guertin,ChristineA;Stevenson,RyanL;Sweeney,JosephD
背景获得性因子(F)XIII缺乏症是一种非常罕见的出血性疾病，具有潜在的致命结局，以前在自身免疫性疾病和白血病的背景下描述过。关于自身抗体的特征和抗纤溶治疗在患者处理中的作用的信息很少。CASE报告一名79岁女性，有3个月的瘀伤和大量月经过多的病史，表现为持续的阴道出血、症状性贫血和右侧大腿血肿。最初的治疗包括腋窝淋巴结活检和凝血评估。活检标本的病理检查显示套细胞淋巴瘤。凝块溶解试验与FXIII活性一致，FXIII活性低于3%。通过微肽阵列分析将其表位特异性定位于FXIII-A亚单位的β-夹心区和催化核心区。冷沉淀、类固醇、利妥昔单抗和抗纤溶治疗的治疗解决了出血的性质并抑制了抑制物。结论这是第一例获得性FXIII抑制物与套细胞淋巴瘤相关的病例，其中准确定义了病理性自身抗体的表位特异性。在免疫抑制开始至病理性抗FXIII自身抗体消失的窗口期，抗纤溶治疗在预防出血并发症中起着重要作用。
BACKGROUNDAcquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management.CASE REPORTA 79‐year‐old woman with a 3‐month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma. Initial management included an axillary lymph node biopsy and coagulation evaluation. Pathologic examination of the biopsy specimen revealed mantle cell lymphoma. Clot solubility assay was consistent with a FXIII activity of less than 3%. An anti‐FXIII inhibitor was suspected, the epitope specificity of which was mapped by micropeptide array analysis to regions in the β‐sandwich and catalytic core domain of the FXIII‐A subunit. Management with cryoprecipitate, steroids, rituximab, and antifibrinolytic therapy resolved the bleeding diathesis and suppressed the inhibitor.CONCLUSIONThis is the first reported case of an acquired FXIII inhibitor associated with mantle cell lymphoma in which the epitope specificity of the pathologic autoantibody was accurately defined. Antifibrinolytic therapy played a prominent role in the prevention of bleeding complications in the window period between initiation of immunosuppression and disappearance of the pathologic anti‐FXIII autoantibody.