Zhang Ya, Xu Fang, Wen Jing-Jing, Shi Lin, Zhou Qiao-Lin
Department of Hematology, Mianyang Central Hospital, Mianyang 621000, Sichuan Province, China.
World J Clin Cases. 2022 Apr 26;10(12):3822-3827. doi: 10.12998/wjcc.v10.i12.3822.
Multiple myeloma patients usually present with CRAB symptoms (hypercalcemia, renal disease, anemia and bone diseases) as initial manifestations. Bleeding symptoms are less common, most of which result from thrombocytopenia or infiltration of plasmacytoma. Relatively, coagulopathy is not so common, especially isolated coagulopathy without CRAB manifestations, which is very rare. Herein, we report a 54-year old female who was hospitalized for intermittent and recurrent mild oral mucosal hemorrhage without other bleeding symptoms for almost one month or typical myeloma features.
Two months before admission, the patient underwent implantation of a permanent pacemaker due to sick sinus syndrome. Prothrombin time and activated partial thromboplastin time were significantly prolonged. Factor X deficiency was demonstrated to account for the coagulation dysfunction. An M protein peak was shown by serum protein electrophoresis. 26.11% of abnormal plasma cells were detected in bone marrow by flow cytometry, expressing CD38, CD138, CD56 and intracellular immunoglobulin Kappa light chain. Bone marrow biopsy also proved the presence of abnormal plasma cells, but Congo red stain was negative. The patient was finally diagnosed with multiple myeloma IgA-κ type. A literature review indicated that factor X deficiency was highly related to amyloidosis. Before bleeding signs, the patient had cardiac arrhythmia, enlargement of the heart, and progressive heart failure; thus, cardiac amyloidosis was suspected.
Bleeding related to coagulation dysfunction is uncommon in multiple myeloma, especially as the initial manifestation. Amyloidosis is a well-recognized cause of isolated acquired factor X deficiency.
多发性骨髓瘤患者通常以CRAB症状(高钙血症、肾脏疾病、贫血和骨病)为初始表现。出血症状较少见,大多由血小板减少或浆细胞瘤浸润所致。相对而言,凝血病并不常见,尤其是无CRAB表现的孤立性凝血病,极为罕见。在此,我们报告一名54岁女性,因间歇性反复轻度口腔黏膜出血住院近1个月,无其他出血症状及典型骨髓瘤特征。
入院前2个月,患者因病态窦房结综合征植入永久性起搏器。凝血酶原时间和活化部分凝血活酶时间显著延长。证实为X因子缺乏导致凝血功能障碍。血清蛋白电泳显示有M蛋白峰。流式细胞术检测骨髓中异常浆细胞占26.11%,表达CD38、CD138、CD56及细胞内免疫球蛋白κ轻链。骨髓活检也证实存在异常浆细胞,但刚果红染色阴性。患者最终诊断为IgA-κ型多发性骨髓瘤。文献回顾表明,X因子缺乏与淀粉样变性高度相关。在出现出血征象前,患者有心律失常、心脏扩大及进行性心力衰竭,因此怀疑有心脏淀粉样变性。
凝血病相关出血在多发性骨髓瘤中不常见,尤其是作为初始表现时。淀粉样变性是公认的孤立性获得性X因子缺乏的病因。