A Case of Refractory Langerhans Cell Histiocytosis Complicated with Hemophagocytic Lymphohistiocytosis Rescued by Cord Blood Transplantation with Reduced-intensity Conditioning

作者：Washio Kana; Muraoka Michiko; Kanamitsu Kiichiro; Oda Megumi; Shimada Akira

来源：Acta Medica Okayama, 2017, 71(3): 249-254.

• langerhans cell histiocytosis (LCH)
• hemophagocytic lymphohistiocytosis (HLH)
• hematopoietic stem cell transplantation (HSCT)
• reduced-intensity conditioning (RIC)
• refractory

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.

• 出版日期2017