Onishi Y, Hotta S, Iwamoto H, Kamoshida T, Takahashi A, Imai Y
Department of Internal Medicine, Hitachi General Hospital.
Rinsho Ketsueki. 1998 Aug;39(8):586-92.
A 66-year-old Japanese man was admitted to Hitachi General Hospital because of fatigue, fever, edema, and icterus. A blood examination showed anemia, leukocytosis, and hyperbilirubinemia. Antibiotics did not alleviate the inflammatory symptoms. On the 13th hospital day, the patient demonstrated mental confusion, with progressive anemia and thrombocytopenia. Bone marrow aspiration revealed an increase of macrophages showing erythrophagocytosis. A diagnosis of hemophagocytic syndrome was made, but no viral infection or hematological malignancy could be detected. The patient was treated with gamma-globulin and methylprednisolone for hemophagocytic syndrome, but died of respiratory insufficiency with progressive hyperbilirubinemia and trombocytopenia on the 29th hospital day, A postmortem examination showed proliferation of lymphoma cells within the small blood vessels of the brain, lungs, liver, and many other organs, but the bone marrow was not involved. An immunohistochemical examination resulted in a diagnosis of intravascular malignant lymphomatosis (IML) of B cell origin. No viral infection was detected with in situ hybridization methods. Although IML occasionally accompanies thrombocytopenia, infiltration of lymphoma cells in the bone marrow is rare. Lymphoma associated hemophagocytic syndrome may cause cytopenia in some patients with IML, as indicated in this case.
一名66岁的日本男性因疲劳、发热、水肿和黄疸入住日立综合医院。血液检查显示贫血、白细胞增多和高胆红素血症。抗生素未能缓解炎症症状。在住院第13天，患者出现精神错乱，伴有进行性贫血和血小板减少。骨髓穿刺显示巨噬细胞增多，可见噬血细胞现象。诊断为噬血细胞综合征，但未检测到病毒感染或血液系统恶性肿瘤。患者接受了γ-球蛋白和甲泼尼龙治疗噬血细胞综合征，但在住院第29天因进行性高胆红素血症和血小板减少导致呼吸功能不全死亡。尸检显示脑、肺、肝和许多其他器官的小血管内有淋巴瘤细胞增殖，但骨髓未受累。免疫组化检查诊断为B细胞起源的血管内恶性淋巴瘤（IML）。原位杂交方法未检测到病毒感染。虽然IML偶尔伴有血小板减少，但淋巴瘤细胞浸润骨髓很少见。如本病例所示，淋巴瘤相关噬血细胞综合征可能导致一些IML患者出现血细胞减少。
