Washio Kana, Muraoka Michiko, Kanamitsu Kiichiro, Oda Megumi, Shimada Akira
Department of Pediatrics, Okayama University Hospital, Okayama 700-8558, Japan.
Acta Med Okayama. 2017 Jun;71(3):249-254. doi: 10.18926/AMO/55208.
We diagnosed a female infant with Langerhans cell histiocytosis (LCH) who was refractory to conventional chemotherapy. She showed refractory inflammation that was complicated with hemophagocytic lymphohistiocytosis (HLH) during LCH chemotherapy; therefore, we changed the protocol to HLH2004 (dexamethasone, cyclosporine A and VP16). However, there were no signs of hematological recovery. We therefore performed cord blood transplantation with reduced-intensity conditioning, and she achieved complete remission for over 2 years. As salvage therapy for refractory LCH, hematopoietic stem cell transplantation may be a good therapeutic choice, especially when LCH is complicated with HLH.
我们诊断出一名患有朗格汉斯细胞组织细胞增多症（LCH）的女婴，她对传统化疗无效。在LCH化疗期间，她出现了难治性炎症，并并发噬血细胞性淋巴组织细胞增生症（HLH）；因此，我们将治疗方案改为HLH2004（地塞米松、环孢素A和VP16）。然而，没有血液学恢复的迹象。因此，我们进行了低强度预处理的脐血移植，她实现了超过2年的完全缓解。作为难治性LCH的挽救治疗，造血干细胞移植可能是一个很好的治疗选择，尤其是当LCH并发HLH时。
