Hyodo T, Iwamasa K, Nakatani S, Yanagisawa K, Hasegawa H, Yasukawa M, Fujita S
First Department of Internal Medicine, Ehime University School of Medicine.
Rinsho Ketsueki. 1998 Sep;39(9):680-5.
An 84-year-old woman was admitted to Ehime University hospital because of fever and generalized swelling of lymph nodes. On admission, she did not have any bone diseases. The laboratory data included a WBC count of 60,900/microliter, with 80.5% atypical plasma cells in the peripheral blood and 26.4% in the bone marrow. The patient's serum total protein was 9.3 g/dl with increased polyclonal gamma-globulin (62.4%). Serum levels of LDH (1,986 IU/l) and IL-6 (34.3 pg/dl) were also elevated. Immunofixation-electrophoresis detected a monoclonal band defined as IgA-lambda type, with a broad band of polyclonal immunoglobulin. Southern blotting analysis demonstrated rearranged monoclonal bands in the JH and J lambda genes. Based on these findings, plasma cell leukemia (IgA-lambda type) was diagnosed. The patient was treated with combination chemotherapy for acute lymphocytic leukemia and achieved complete remission. However, she died of aspergillus sepsis two months after admission. After chemotherapy, the patients IgG and IgM levels normalized but IgA still showed a slight increase. IL-6 also decreased, from 34.3 pg/dl to 10.2 pg/dl. To our knowledge, this report is the first in the literature concerning a case of plasma cell leukemia with polyclonal hypergammaglobulinemia.
一名84岁女性因发热和全身淋巴结肿大入住爱媛大学医院。入院时，她没有任何骨骼疾病。实验室检查数据包括白细胞计数为60,900/微升，外周血中非典型浆细胞占80.5%，骨髓中占26.4%。患者血清总蛋白为9.3g/dl，多克隆γ球蛋白升高（62.4%）。血清乳酸脱氢酶（LDH）水平（1,986IU/l）和白细胞介素-6（IL-6）水平（34.3pg/dl）也升高。免疫固定电泳检测到一条定义为IgA-λ型的单克隆条带，伴有多克隆免疫球蛋白的宽带。Southern印迹分析显示JH和Jλ基因中有重排的单克隆条带。基于这些发现，诊断为浆细胞白血病（IgA-λ型）。该患者接受了急性淋巴细胞白血病联合化疗并实现完全缓解。然而，她在入院两个月后死于曲霉菌败血症。化疗后，患者的IgG和IgM水平恢复正常，但IgA仍略有升高。IL-6也从34.3pg/dl降至10.2pg/dl。据我们所知，本报告是文献中首例关于伴有多克隆高丙种球蛋白血症的浆细胞白血病病例。
