Hasegawa D, Sano K, Kosaka Y, Hayakawa A, Nakamura H
Department of Pediatrics, Kobe University School of Medicine, Kobe, Japan.
Bone Marrow Transplant. 1999 Aug;24(4):425-7. doi: 10.1038/sj.bmt.1701917.
We report a 7-year-old girl with hemophagocytic lymphohistiocytosis who received a syngeneic bone marrow transplant from her twin sister. She presented with high fever and cough. Laboratory findings revealed pancytopenia, elevation of liver enzymes, and hyperferritinemia. Bone marrow examination revealed histiocytic hemophagocytes and lymphoblastoid cells. Southern blot analysis of the bone marrow cells revealed a monoclonal proliferation of EBV-infected lymphocytes. Although she underwent combined chemotherapy according to the HLH-94 protocol, she developed severe pancytopenia. Following myeloablative conditioning with busulfan (16 mg/kg), cyclophosphamide (120 mg/kg), and etoposide (1.5 g/m2), she was transplanted with 6.6 x 10(8)/kg mononuclear cells from the twin sister. She remains in complete remission 23 months after transplantation.
我们报告了一名7岁患有噬血细胞性淋巴组织细胞增生症的女孩，她接受了来自其双胞胎姐姐的同基因骨髓移植。她表现为高热和咳嗽。实验室检查发现全血细胞减少、肝酶升高和高铁蛋白血症。骨髓检查发现组织细胞性噬血细胞和淋巴母细胞样细胞。对骨髓细胞进行Southern印迹分析显示EB病毒感染的淋巴细胞呈单克隆增殖。尽管她按照HLH - 94方案接受了联合化疗，但仍出现严重的全血细胞减少。在用白消安（16 mg/kg）、环磷酰胺（120 mg/kg）和依托泊苷（1.5 g/m²）进行清髓预处理后，她接受了来自双胞胎姐姐的6.6 x 10(8)/kg单个核细胞移植。移植后23个月她仍处于完全缓解状态。
