Harada Shuko, Kalla Hari, Balasubramanian Manjula, Brodsky Isadore, Gladstone Douglas, Hou J Steve
Department of Pathology and Laboratory Medicine, Drexel University College of Medicine, Philadelphia, PA 19102, USA.
Ann Diagn Pathol. 2008 Jun;12(3):212-6. doi: 10.1016/j.anndiagpath.2006.12.005. Epub 2007 Sep 14.
Combination of the splenic marginal zone B-cell lymphoma (SMZL) and classical Hodgkin lymphoma (cHL) is extremely rare. We report a unique case with concurrent SMZL and cHL. The patient was a 63-year-old man who presented with fatigue and anemia, showing a splenomegaly and retroperitoneal lymphadenopathy. A splenectomy revealed monotonous marginal zone lymphocytic infiltrates and numerous large Reed-Sternberg-like cells. Flow cytometry revealed a kappa light-chain-restricted CD5 (-), CD23 (-) B-cell population. DNA polymerase chain reaction analysis confirmed the presence of clonal rearrangement of the immunoglobulin heavy-chain gene. Immunohistochemical studies revealed that the large atypical cells were CD30 (+), CD15 (weakly +), CD20 (-), CD45 (-), Pax5 (weakly +), BOB.1 (-), and Oct2 (-), indicating the coexistence of SMZL with cHL. After the chemotherapy, the patient achieved a clinical/radiologic remission, whereas cHL was detected in liver and bone marrow subsequently. The case indicates that both components of lymphoma can present concurrently as a composite form of lymphoma and both need to be treated adequately.
脾边缘区B细胞淋巴瘤（SMZL）与经典型霍奇金淋巴瘤（cHL）的合并极为罕见。我们报告了一例同时患有SMZL和cHL的独特病例。患者为一名63岁男性，表现为疲劳和贫血，伴有脾肿大和腹膜后淋巴结病。脾切除术显示有单调的边缘区淋巴细胞浸润以及众多大的里德-斯腾伯格样细胞。流式细胞术显示kappa轻链受限的CD5（-）、CD23（-）B细胞群。DNA聚合酶链反应分析证实存在免疫球蛋白重链基因的克隆重排。免疫组织化学研究显示，大的非典型细胞CD30（+）、CD15（弱阳性）、CD20（-）、CD45（-）、Pax5（弱阳性）、BOB.1（-）和Oct2（-），表明SMZL与cHL共存。化疗后，患者实现了临床/影像学缓解，但随后在肝脏和骨髓中检测到了cHL。该病例表明，淋巴瘤的两种成分可同时以淋巴瘤的复合形式出现，且两者均需得到充分治疗。
