Richter J, Swedin A, Olofsson T, Johansson B, Akerman M, Winqvist I
Department of Medicine, University Hospital, Lund, Sweden.
Ann Hematol. 1995 Dec;71(6):307-10. doi: 10.1007/BF01697984.
A case of aggressive plasma cell leukemia with unusual morphological and cytogenetic features is reported. A 65-year-old man was admitted to hospital due to anemia, thrombocytopenia, and renal insufficiency. Bone marrow examination and peripheral blood smear revealed a large number of pleomorphic cells with convoluted and multilobulated nuclei. Immunohistochemistry of the bone marrow biopsy was negative for anti-keratin antibodies CAM.5.2 and AE1/AE3, but positive for EMA. The immunophenotypic features of these cells were suggestive of plasma cell origin with positivity for CD38, CD56, CD9, and CD44 and a weak positivity for CD71 and CD45 (40% of the cells), while all other markers of hematopoietic origin were negative. Furthermore, a serum protein electrophoresis showed a monoclonal component type IgG-kappa of 70 g/l. The cytogenetic analysis demonstrated a hypotetraploid clone with multiple numerical and structural abnormalities. Although some of the aberrations found are associated with plasma cell malignancies--e.g., structural rearrangement of chromosome 1, del(6q), and monosomy 13--the karyotypic complexity in the present case is unusual. The course of the disease was very aggressive, and the patient died 3 days after admission.
报告了一例具有不寻常形态学和细胞遗传学特征的侵袭性浆细胞白血病病例。一名65岁男性因贫血、血小板减少和肾功能不全入院。骨髓检查和外周血涂片显示大量多形性细胞，核呈卷曲状和分叶状。骨髓活检的免疫组织化学检测显示抗角蛋白抗体CAM.5.2和AE1/AE3阴性，但EMA阳性。这些细胞的免疫表型特征提示为浆细胞来源，CD38、CD56、CD9和CD44呈阳性，CD71和CD45呈弱阳性（40%的细胞），而所有其他造血来源标志物均为阴性。此外，血清蛋白电泳显示单克隆成分类型为IgG-κ，浓度为70 g/l。细胞遗传学分析显示一个亚四倍体克隆，伴有多种数量和结构异常。虽然所发现的一些畸变与浆细胞恶性肿瘤相关，例如1号染色体结构重排、del(6q)和13号染色体单体，但本例的核型复杂性并不寻常。疾病进展非常迅速，患者入院3天后死亡。
