Langer Sabina, Mehta Meenal, Saraf Amrita, Gupta Aastha, Pipliya Keyur, Kakar Atul, Bhargava Manorama
Department of Hematology, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi, 110060 India.
Department of Medicine, Sir Ganga Ram Hospital, New Delhi, India.
Indian J Hematol Blood Transfus. 2016 Jun;32(Suppl 1):173-7. doi: 10.1007/s12288-015-0512-7. Epub 2015 Mar 1.
A 74 years old male patient, presented with history of generalized weakness, fatigue, loss of appetite and breathlessness on exertion for past one and a half months. On examination, he was found to have significant pallor and generalized lymphadenopathy (cervical, axillary and inguinal). The skeletal survey showed punched out lytic lesions in skull and pelvic bones. The peripheral smear examination showed lymphocytosis with absolute lymphocyte count of 25,000/μL. The bone marrow aspirates revealed a hypercellular marrow with 74 % lymphocytes & 14 % plasma cells, suggestive of chronic lymphoplasmacytic disorder. The bone marrow biopsy had two morphologically distinct populations of lymphocytes & plasma cells. The immunohistochemical markers on bone marrow biopsy showed hat plasma cells were positive for CD138 with kappa light chain restriction. Flow cytometry showed B cell population with CD19/CD5 co expression, CD5/CD23 coexpression, were positive for CD22, CD20 and negative for FMC-7 and lambda light chain. In addition, plasma cells were also identified as CD45 negative cells and showed CD38/CD138 co-expression with variable CD19 and CD56 positivity. Serum protein electrophoresis revealed M band, serum immunofixation electrophoresis corresponded to IgA -Kappa. The final diagnosis of chronic lymphocytic leukemia with concomittant presence of plasma cell myeloma was concluded. This case imparts an important message to look for presence of coexisting entities in a single specimen and highlights the benefits of testing both plasma cell and B-cell compartments when the clinical features are not entirely consistent Flow cytometry together with protein electrophoresis can help to clinch difficult and rare dual diagnosis. These cases are rare and pose therapeutic challenge.
一名74岁男性患者,在过去一个半月中出现全身无力、疲劳、食欲不振和劳力性呼吸困难的病史。检查发现他有明显的面色苍白和全身淋巴结肿大(颈部、腋窝和腹股沟)。骨骼检查显示颅骨和骨盆骨有穿凿样溶骨性病变。外周血涂片检查显示淋巴细胞增多,绝对淋巴细胞计数为25,000/μL。骨髓穿刺显示骨髓细胞增多,淋巴细胞占74%,浆细胞占14%,提示慢性淋巴细胞浆细胞性疾病。骨髓活检有两种形态学上不同的淋巴细胞和浆细胞群体。骨髓活检的免疫组化标记显示浆细胞CD138阳性,kappa轻链受限。流式细胞术显示B细胞群体有CD19/CD5共表达、CD5/CD23共表达,CD22、CD20阳性,FMC-7和lambda轻链阴性。此外,浆细胞也被鉴定为CD45阴性细胞,显示CD38/CD138共表达,CD19和CD56阳性情况各异。血清蛋白电泳显示M带,血清免疫固定电泳对应于IgA-κ。最终诊断为慢性淋巴细胞白血病合并浆细胞骨髓瘤。该病例传达了一个重要信息,即在单个标本中寻找共存实体的存在,并强调当临床特征不完全一致时,检测浆细胞和B细胞区室的益处。流式细胞术与蛋白电泳相结合有助于明确困难和罕见的双重诊断。这些病例罕见且带来治疗挑战。