Bolme P, Henter J I, Winiarski J, Elinder G, Ljungman P, Lönnerholm G, Ringdén O
Division of Pediatrics, Huddinge Hospital, Sweden.
Bone Marrow Transplant. 1995 Mar;15(3):331-5.
Six children (aged 9 months to 10 and 5/12 years) with hemophagocytic lymphohistiocytosis (HLH) have undergone allogeneic BMT in Sweden. In two of the children unrelated donors were used. Parents were available as donors in two of the cases and siblings in the other two. Conditioning before BMT consisted of etoposide, busulfan and cyclophosphamide with the addition of ATG in two cases and OKT 3 in one case. For post-transplant immunosuppression, i.v. methotrexate and cyclosporin A (CsA) were used in five cases, and in one child CsA was combined with methylprednisolone. Of the six children, four are alive and well 2 and 3/12 to 3 and 1/12 years after BMT. One child, who had an unrelated donor with one DR-antigen mismatch, died 30 days after BMT of fulminant grade IV GVHD. Another patients, seropositive for CMV, received marrow from an unrelated HLA-A, -B, -DR and -DP identical donor. After an initially uneventful course, CMV was isolated from her leukocytes. Seven months after BMT she developed a progressive obstructive chronic bronchiolitis and succumbed to respiratory insufficiency 14 months after the transplant. This study supports the view that BMT is the treatment of choice in HLH, particularly if an HLA-identical related donor is available.
在瑞典，6名患有噬血细胞性淋巴组织细胞增生症（HLH）的儿童（年龄从9个月至10岁零5/12岁）接受了异基因骨髓移植（BMT）。其中两名儿童使用了无关供体。在两例中父母作为供体，另外两例中兄弟姐妹作为供体。BMT前的预处理方案包括依托泊苷、白消安和环磷酰胺，两例加用了抗胸腺细胞球蛋白（ATG），一例加用了OKT 3。对于移植后免疫抑制，5例使用了静脉注射甲氨蝶呤和环孢素A（CsA），1名儿童的CsA与甲泼尼龙联合使用。6名儿童中，4名在BMT后2年零3/12至3年零1/12年存活且状况良好。一名儿童使用了一名有一个DR抗原错配的无关供体，在BMT后30天死于暴发性IV级移植物抗宿主病（GVHD）。另一名患者巨细胞病毒（CMV）血清学阳性，接受了一名HLA - A、- B、- DR和 - DP全相合的无关供体的骨髓。在最初一段平稳病程后，从她的白细胞中分离出CMV。BMT后7个月，她出现进行性阻塞性慢性细支气管炎，并在移植后14个月死于呼吸功能不全。这项研究支持了BMT是HLH的首选治疗方法这一观点，特别是如果有HLA全相合的相关供体可用时。
