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Haematopoietic stem cell transplantation for refractory Langerhans cell histiocytosis: outcome by intensity of conditioning
Author(s) -
Veys Paul A.,
Nanduri Vasanta,
Baker K. Scott,
He Wensheng,
Bandini Giuseppe,
Biondi Andrea,
Dalissier Arnaud,
Davis Jeffrey H.,
Eames Gretchen M.,
Egeler R. Maarten,
Filipovich Alexandra H.,
Fischer Alain,
Jürgens Herbert,
Krance Robert,
Lanino Edoardo,
Leung Wing H.,
Matthes Susanne,
Michel Gérard,
Orchard Paul J.,
Pieczonka Anna,
Ringdén Olle,
Schlegel Paul G.,
Sirvent Anne,
Vettenranta Kim,
Eapen Mary
Publication year - 2015
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.13347
Subject(s) - medicine , transplantation , chemotherapy , langerhans cell histiocytosis , refractory (planetary science) , total body irradiation , hematopoietic stem cell transplantation , surgery , cytarabine , oncology , alemtuzumab , stem cell , gastroenterology , cyclophosphamide , disease , biology , genetics , astrobiology
Summary Patients with Langerhans cell histiocytosis ( LCH ) refractory to conventional chemotherapy have a poor outcome. There are currently two promising treatment strategies for high‐risk patients: the first involves the combination of 2‐chlorodeoxyadenosine and cytarabine; the other approach is allogeneic haematopoietic stem cell transplantation ( HSCT ). Here we evaluated 87 patients with high‐risk LCH who were transplanted between 1990 and 2013. Prior to the year 2000, most patients underwent HSCT following myeloablative conditioning ( MAC ): only 5 of 20 patients (25%) survived with a high rate (55%) of transplant‐related mortality ( TRM ). After the year 2000 an increasing number of patients underwent HSCT with reduced intensity conditioning ( RIC ): 49/67 (73%) patients survived, however, the improved survival was not overtly achieved by the introduction of RIC regimens with similar 3‐year probability of survival after MAC (77%) and RIC transplantation (71%). There was no significant difference in TRM by conditioning regimen intensity but relapse rates were higher after RIC compared to MAC regimens (28% vs. 8%, P = 0·02), although most patients relapsing after RIC transplantation could be salvaged with further chemotherapy. HSCT may be a curative approach in 3 out of 4 patients with high risk LCH refractory to chemotherapy: the optimal choice of HSCT conditioning remains uncertain.