Alatoom Adnan, Elsabrouty Rania, Willis Jason, Boils Christie, Sarode Ravindra, Hashim Ibrahim, Wang Huan-You
Department of Pathology, the University of Texas Southwestern Medical Center at Dallas 5323 Harry Hines Blvd, Dallas, Texas, USA.
Int J Clin Exp Pathol. 2009;2(5):498-507. Epub 2009 Feb 9.
IgM plasma cell myeloma (PCM) is a rare entity, and CD19 positivity is found in only 1-4% of PCM. Here we report a unique case of IgM PCM, in which the plasma cells are positive for CD19. Clinically, the patient presented with hyperviscosity syndrome, mimicking the clinical manifestation of Waldenstrom's macroglobulinemia. In addition, the IgM para-protein from the patient behaved like cryoglobulins, which interfered with some of the laboratory measurements and resulted in erroneous platelet count, mean platelet volume, and serum IgM level. Despite chemotherapy, the PCM persisted and progressed to plasma cell leukemia, and the patient died of a left frontal hematoma with widespread cerebral hemorrhage extending from left lateral ventricle, third ventricle, fourth ventricle, to cisterna magna. This case represents the first CD19+ IgM-producing PCM and the second case of brain hemorrhage due to plasma cell myeloma/leukemia.
IgM 浆细胞骨髓瘤（PCM）是一种罕见的疾病，仅1% - 4%的PCM病例中可发现浆细胞CD19呈阳性。在此，我们报告一例独特的IgM PCM病例，其浆细胞CD19呈阳性。临床上，该患者表现为高黏滞综合征，类似于华氏巨球蛋白血症的临床表现。此外，该患者的IgM副蛋白表现为冷球蛋白，干扰了一些实验室检测结果，导致血小板计数、平均血小板体积及血清IgM水平出现误差。尽管接受了化疗，PCM仍持续存在并进展为浆细胞白血病，患者最终死于左额叶血肿，脑出血广泛，从左侧脑室、第三脑室、第四脑室延伸至枕大池。该病例是首例产生IgM的CD19阳性PCM，也是第二例因浆细胞骨髓瘤/白血病导致脑出血的病例。
