Balkis Trabelsi, Hela Baccouche, Jannet Eddhib, Aya Chakroun, Mahjoub Sonia
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引用次数: 0
摘要
本病例涉及一名 58 岁的患者,无个人或家族出血史,2016 年确诊为 III-A 期 IgG Kappa 多发性骨髓瘤,2017 年接受了自体移植手术。2022 年 3 月,他的骨髓瘤复发。2022 年 10 月,患者出现左肩和血胸腔大面积自发性压迫性血肿,需要进行两次手术治疗。血小板计数和止血指标均在正常范围内。进一步的诊断检测显示患者患有获得性 XIII 因子缺乏症和获得性血栓病。由于缺乏针对因子 XIII B 亚基的特异性检测方法,诊断工作面临挑战。治疗包括输血和皮质类固醇,但疗效有限。患者对化疗的反应引发了单克隆成分是否参与其中的疑问。随后的随访显示,即使没有症状,XIII因子的水平也在波动。由于出血风险很大,患者被认为不符合自体移植的条件。
Acquired factor XIII deficiency and its unprecedented association with multiple myeloma: case report and literature review.
This case involves a 58-year-old patient, with no personal or family history of bleeding, diagnosed with stage III-A IgG Kappa multiple myeloma in 2016 and underwent autografting in 2017. In March 2022, he suffered a myeloma relapse. In October 2022, the patient presented with a large spontaneous compressive hematoma in the left shoulder and hemithorax, requiring two surgical procedures. The platelet count and hemostasis parameters were within normal range. Further diagnostic testing revealed acquired factor XIII deficiency and acquired thrombopathy. Diagnostic challenges arose from the absence of specific assays for the B subunit of Factor XIII. Treatment involved transfusions and corticosteroids, but efficacity was limited. The patient's response to chemotherapy raised questions about the involvement of the monoclonal component. Subsequent follow-ups showed factor XIII levels fluctuating, even without symptoms. The patient was deemed ineligible for autograft due to a significant risk of hemorrhage.
期刊介绍:
Blood Coagulation & Fibrinolysis is an international fully refereed journal that features review and original research articles on all clinical, laboratory and experimental aspects of haemostasis and thrombosis. The journal is devoted to publishing significant developments worldwide in the field of blood coagulation, fibrinolysis, thrombosis, platelets and the kininogen-kinin system, as well as dealing with those aspects of blood rheology relevant to haemostasis and the effects of drugs on haemostatic components