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

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

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 (dexamet...

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Veröffentlicht in:	Acta medica Okayama 2017-06, Vol.71 (3), p.249
Hauptverfasser:	Washio, Kana, Muraoka, Michiko, Kanamitsu, Kiichiro, Oda, Megumi, Shimada, Akira
Format:	Artikel
Sprache:	eng
Schlagworte:	Antineoplastic Combined Chemotherapy Protocols - therapeutic use Cord Blood Stem Cell Transplantation - methods Female Histiocytosis, Langerhans-Cell - complications Histiocytosis, Langerhans-Cell - pathology Histiocytosis, Langerhans-Cell - therapy Humans Infant Lymphohistiocytosis, Hemophagocytic - complications Lymphohistiocytosis, Hemophagocytic - pathology Lymphohistiocytosis, Hemophagocytic - therapy Salvage Therapy Tomography, X-Ray Computed Transplantation Conditioning - methods Treatment Outcome
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Zusammenfassung:	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.
ISSN:	0386-300X