Document Detail


Hematopoietic stem cell transplantation in patients with severe Langerhans cell histiocytosis and hematological dysfunction: experience of the French Langerhans Cell Study Group.
MedLine Citation:
PMID:  12796789     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The aim of this study was to assess the results of hematopoietic stem cell transplantation (HSCT) in refractory Langerhans cell histiocytosis (LCH). Among 85 patients with LCH and hematological dysfunction diagnosed in France between 1987 and 2000, eight received HSCT in six institutions. Median age at diagnosis was 0.54 years. The median LCH activity score at diagnosis was 10 (range 3-20). All patients responded poorly to initial chemotherapy. At the time of HSCT, the median activity score was 16.5 (range 7-18). HSCT was autologous in three cases and allogeneic in five cases. The conditioning regimen consisted of TBI in two cases and chemotherapy alone in six cases. Conditioning had to be attenuated in two patients. All patients had persistent active disease after autologous HSCT, which was fatal in two cases and controlled by chemotherapy in one case. After allogeneic HSCT, two patients died from toxicity and three had complete responses; two patients had had no recurrences after 21 months and 7 years of follow-up, while the other patient relapsed and died from sepsis related to splenectomy. HSCT for refractory LCH can thus be highly toxic but can also achieve sustained disease control.
Authors:
V Akkari; J Donadieu; C Piguet; P Bordigoni; G Michel; S Blanche; J L Casanova; C Thomas; E Vilmer; A Fischer; Y Bertrand;
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Bone marrow transplantation     Volume:  31     ISSN:  0268-3369     ISO Abbreviation:  Bone Marrow Transplant.     Publication Date:  2003 Jun 
Date Detail:
Created Date:  2003-06-10     Completed Date:  2004-03-01     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8702459     Medline TA:  Bone Marrow Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  1097-103     Citation Subset:  IM    
Affiliation:
Service d'hématologie Pédiatrique, Hôpital Debrousse, 29 rue Soeur Bouvier Lyon, France.
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MeSH Terms
Descriptor/Qualifier:
Chimera
Female
France
Graft Survival
Graft vs Host Disease / etiology
Hematopoietic Stem Cell Transplantation* / adverse effects
Histiocytosis, Langerhans-Cell / therapy*
Humans
Infant
Male
Prognosis
Prospective Studies
Retrospective Studies
Transplantation Conditioning
Transplantation, Autologous
Transplantation, Homologous

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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