Sari Ismail, Erkurt Mehmet Ali, Ifran Ahmet, Kaptan Kursat, Beyan Cengiz
Department of Hematology, Pamukkale University Faculty of Medicine, 20070, Denizli, Turkey.
Department of Hematology, Gulhane Military Medical Academy, Etlik, Ankara, Turkey.
Int J Hematol. 2009 Sep;90(2):166-169. doi: 10.1007/s12185-009-0363-9. Epub 2009 Jun 24.
An initial presentation of hematological malignancies associated with autoantibodies is not common, and there is only one documented case of multiple myeloma presenting with acquired FVIII inhibitor for multiple myeloma. In this paper, we describe a second case of multiple myeloma who presented with acquired FVIII inhibitor. A 43-year-old woman was referred to our hematology unit for anemia and an elevated erythrocyte sedimentation rate. Two months before her admission, she had undergone an operation at a local hospital because of ovarian cyst rupture complicated by severe postoperative bleeding. Because coagulation tests had revealed a prolonged partial thromboplastin time which could not be corrected by a mixing test and a decreased FVIII level, a diagnosis of acquired FVIII inhibitor had been made. The patient was hospitalized in our unit for further evaluation. The erythrocyte sedimentation rate was 110 mm/h, serum albumin level 2.5 g/dL, globulin level 5.6 g/dL, and C-reactive protein 47.8 mg/L (0-6). Serum IgG was high, and serum protein electrophoresis showed a monoclonal spike in the gamma region. An IgG-kappa paraprotein was identified by immunofixation of the urine and serum. X-ray films of the bones revealed lytic areas in the skull, pelvis, and lumbar vertebrae. Bone marrow aspiration showed normal cellularity with 40% plasma cell infiltration. The patient was diagnosed with the IgG kappa type of multiple myeloma associated with acquired FVIII inhibitor. In patients presenting with severe bleeding, autoantibodies against FVIII should be considered for the differential diagnosis of bleeding. Clinicians should be alert to the presence of rare underlying neoplastic diseases such as multiple myeloma, in patients with acquired FVIII inhibitor.
血液系统恶性肿瘤与自身抗体相关的初始表现并不常见,仅有一例记录在案的多发性骨髓瘤患者出现获得性FVIII抑制剂。在本文中,我们描述了第二例出现获得性FVIII抑制剂的多发性骨髓瘤患者。一名43岁女性因贫血和红细胞沉降率升高被转诊至我们的血液科。入院前两个月,她因卵巢囊肿破裂在当地医院接受手术,术后出现严重出血。由于凝血检查显示部分凝血活酶时间延长,混合试验无法纠正,且FVIII水平降低,故诊断为获得性FVIII抑制剂。患者入住我们科室进行进一步评估。红细胞沉降率为110mm/h,血清白蛋白水平为2.5g/dL,球蛋白水平为5.6g/dL,C反应蛋白为47.8mg/L(0 - 6)。血清IgG升高,血清蛋白电泳显示γ区有单克隆峰。通过尿液和血清免疫固定鉴定出IgG-κ副蛋白。骨骼X线片显示颅骨、骨盆和腰椎有溶骨性区域。骨髓穿刺显示细胞数量正常,浆细胞浸润率为40%。该患者被诊断为IgG κ型多发性骨髓瘤合并获得性FVIII抑制剂。对于出现严重出血的患者,应考虑针对FVIII的自身抗体以进行出血的鉴别诊断。临床医生应警惕在获得性FVIII抑制剂患者中存在罕见的潜在肿瘤性疾病,如多发性骨髓瘤。