Primary Bone Marrow Large B-cell Lymphoma Presenting with Hemophagocytic Lymphohistiocytosis

原發骨髓淋巴癌是一種罕見的淋巴癌，合併噬血症候群等少見的臨床表現經常延誤正確即時的診斷和治療。我們報告一名76歲女性因間斷發燒、黃疸、肝脾腫大、貧血及血小板低下兩週前來就診，經給予抗生素後仍持續發燒，全身電腦斷層顯示肝脾腫大，但無異常淋巴結腫大情形。骨髓檢查確診為大B細胞淋巴癌（large B cell lymphoma）併噬血症候群，經使用標靶併化學治療（rituximab, cyclophosphamide, vincristine, prednisolone），發燒、黃疸、血球異常等症狀均恢復正常，病患經完成六次化學治療及追蹤十八個月後，目前疾病仍處於完全緩解狀態。在不明原因發燒合併血球低下的病患，臨床醫師應即時進行骨髓檢查，並懷疑有原發骨髓淋巴癌之可能。

關鍵字

原發骨髓大B細胞淋巴癌；原發骨髓淋巴癌；非何杰金氏淋巴癌；噬血症候群；噬血淋巴組織球增生症

並列摘要

Primary bone marrow lymphoma is an extremely rare disease. Its unusual clinical manifestations, such as hemophagocytic syndrome, frequently delay correct disease diagnosis, thus postponing treatment. Here, we reported a 76-year-old woman presenting with intermittent night fever and jaundice for 2 weeks, accompanied with hepatosplenomegaly and pancytopenia. Despite antibiotics treatment, the patient’s fever persisted. Computed tomography showed hepatosplenomegaly without lymphadenopathy, and bone marrow aspiration and biopsy revealed a large B-cell lymphoma (LBCL) with hemophagocytic lymphohistiocytosis (HLH). After chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisolone, both jaundice and pancytopenia recovered to normal. After 6 cycles of chemotherapy, the patient remained in complete remission for 18 months after diagnosis. Our experience indicates that clinicians should consider performing a timely bone marrow examination on patients with unknown fever and pancytopenia, particularly given that delayed diagnosis of primary bone marrow lymphoma can make treatment of this rare disease substantially more challenging.

並列關鍵字

primary bone marrow large B-cell lymphoma ； non-Hodgkin's lymphoma ； hemophagocytic lymphohistiocytosis ； hemophagocytic syndrome

國際替代計量

Primary Bone Marrow Large B-cell Lymphoma Presenting with Hemophagocytic Lymphohistiocytosis

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