Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis
Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis
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DOI：
10.1038/nrclinonc.2010.40
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发表时间：
2010-07-01
影响因子：
78.8
通讯作者：
Berliner, Nancy
中科院分区：
文献类型：
--
作者：
Strout, Matthew P.;Seropian, Stuart;Berliner, Nancy
背景资料。一名39岁的女性，没有相关的病史或家族史，因发烧入院，尽管接受了抗生素治疗，但仍持续发烧。入院时其他值得注意的发现包括脾肿大、全血细胞减少、低钠血症、肝酶水平升高、高铁蛋白血症和低纤维蛋白原血症。体检、实验室检查、风湿标志物血清学、病原体检测、全血计数、铁蛋白、纤维蛋白原、甘油三酯和可溶性CD25水平的测定、自然杀伤细胞功能研究、PRF1突变分析、肾活检、骨髓活检、胸部和腹部的CT成像。特发性，非典型噬血细胞性淋巴组织细胞增多症。首先使用抗生素治疗，然后进行免疫抑制治疗(包括静脉注射免疫球蛋白、环孢素、英夫利昔单抗、皮质类固醇和依托泊苷)。缓解是通过使用抗CD52的单抗alemtuzumab，然后异基因干细胞移植(结合降低强度的条件化治疗和移植物抗宿主病预防)导致明确的治愈。
Background. A 39-year-old woman with no relevant medical or family history was admitted to hospital with episodic fever, which persisted despite antibiotic therapy. Other notable findings at admission were splenomegaly, pancytopenia, hyponatremia, elevated levels of liver enzymes, hyperferritinemia and hypofibrinogenemia.Investigations. Physical examination, laboratory tests, rheumatic marker serology, pathogen detection assays, complete blood counts, measurement of levels of ferritin, fibrinogen, triglycerides and soluble CD25, natural killer cell functional studies, PRF1 mutation analysis, renal biopsy, bone marrow biopsy, CT imaging of the chest and abdomen.Diagnosis. Idiopathic, atypical hemophagocytic lymphohistiocytosis.Management. Initial treatment with antibiotics was followed by immunosuppressive therapy (including intravenous immunoglobulin, ciclosporin, infliximab, corticosteroids and etoposide). Remission was achieved by treatment with the anti-CD52 monoclonal antibody, alemtuzumab, after which allogeneic stem-cell transplantation (with reduced-intensity conditioning treatment and graft-versus-host disease prophylaxis) resulted in a definitive cure.