Masutani K, Fujimaru M, Tsubota Y, Matsui N, Okamura T
Division of Internal Medicine, Kyushu Central Hospital.
Rinsho Ketsueki. 1997 Nov;38(11):1199-205.
A 79-year-old woman was admitted with general fatigue, jaundice and hepatosplenomegaly. Perpheral blood examination showed 8.0 g/dl Hb, 15 x 10(3)/microliter platelet and 10,490/microliter leukocytes with 86% abnormal lymphocytes. Immunophenotypic analysis of abnormal lymphocytes demonstrated CD2(+), CD3(+/-), CD4(-), and CD8(-). Serum antibody for HTLV-1 was positive. In addition, the monoclonal integration of HTLV-1 proviral DNA into the genome of leukemic cells was demonstrated on Southern blot hybridization. Bone marrow revealed ATL cell in vasion with myelofibrosis and hemophagocytic cell proliferation. Therefore, adult T-cell leukemia with hemophagocytic syndrome was diagnosed. She was treated with methyl prednisolone pulse therapy and gammaglobulin. But she died of hepatic failure 14 days after hospitalization. On autopsy, EB virus LMP-1 was detected in ATL cells in bone marrow. ATL with hemophagocytosis is relatively rare. The association of both pathological states was discussed.
一名79岁女性因全身乏力、黄疸和肝脾肿大入院。外周血检查显示血红蛋白8.0 g/dl，血小板15×10³/微升，白细胞10490/微升，其中86%为异常淋巴细胞。对异常淋巴细胞的免疫表型分析显示CD2(+)、CD3(+/-)、CD4(-)和CD8(-)。HTLV-1血清抗体呈阳性。此外，Southern印迹杂交证实HTLV-1前病毒DNA单克隆整合到白血病细胞基因组中。骨髓检查显示成人T细胞白血病细胞浸润伴骨髓纤维化和噬血细胞增殖。因此，诊断为成人T细胞白血病合并噬血细胞综合征。给予甲基强的松龙冲击治疗和丙种球蛋白治疗。但患者住院14天后死于肝衰竭。尸检时，在骨髓中的成人T细胞白血病细胞中检测到EB病毒LMP-1。成人T细胞白血病合并噬血细胞现象相对罕见。对这两种病理状态的关联进行了讨论。
