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Cytologic Diagnosis of Primary Effusion Lymphoma in an HIV-Negative Patient

以細胞學診斷人類免疫缺陷病毒陰性之原發性積液淋巴瘤

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摘要


原發性積液淋巴瘤是一種罕見的非何杰金淋巴瘤。不同於其他淋巴瘤多以器官或淋巴結腫大來表現,其特徵是含有淋巴瘤的體液在體腔內(如腹腔、胸腔、心包膜腔)異常聚積,而患者多有8型人類皰疹病毒(HHV-8)感染相關或好發在免疫不全的人身上。我們報告一位69歲男性以腹腔、胸腔積水作表現的原發性積液淋巴瘤的病例。原發性積液淋巴瘤的細胞型態為單形性非典型性淋巴組織增生,具有中型到大型的單核或雙核及少量到中量有空泡的嗜鹹性細胞質。免疫染色方面,這些非典型性淋巴球表現CD138、MUM1及HHV-8。並作PCR檢定其為單株細胞免疫蛋白重鏈基因重組而原位雜交顯示為Epstein-Barr病毒陰性。不幸,此患者於確診後在接受第一次化學治療的過程中死亡。

並列摘要


Primary effusion lymphoma (PEL) is an unusual and rare type of non-Hodgkin's lymphoma characterized by lymphomatous effusion of pleural, pericardial or peritoneal cavities without lymphadenopathy or organomegaly. It is associated with human herpes virus-8 (HHV-8) and occurs most often in immunodeficient patients. We present a case of PEL in a 69-year-old male presenting with pleural effusion and ascites. Fluid aspiration showed a monomorphic population of atypical lymphoid cells, which were medium-to large-sized, with mono- or binucleated hyper-chromatic nuclei and a small to moderate amount of basophilic cytoplasm containing cytoplasmic vesicles. Immunohistochemically, the lymphoid cells expressed CD138 and multiple myeloma oncogene 1, were positive for HHV-8, and were monoclonal for immunoglobulin heavy chain gene rearrangement. They were negative for Epstein-Barr virus by in situ hybridization. Unfortunately, the patient died during the first course of chemotherapy with cyclophosphamide, vincristine and prednisone.

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