Title |
Haematopoietic stem cell transplantation for refractory Langerhans cell histiocytosis: outcome by intensity of conditioning
|
---|---|
Published in |
British Journal of Haematology, March 2015
|
DOI | 10.1111/bjh.13347 |
Pubmed ID | |
Authors |
Paul A Veys, Vasanta Nanduri, K Scott Baker, Wensheng He, Giuseppe Bandini, Andrea Biondi, Arnaud Dalissier, Jeffrey H Davis, Gretchen M Eames, R Maarten Egeler, Alexandra H Filipovich, Alain Fischer, Herbert Jürgens, Robert Krance, Edoardo Lanino, Wing H Leung, Susanne Matthes, Gérard Michel, Paul J Orchard, Anna Pieczonka, Olle Ringdén, Paul G Schlegel, Anne Sirvent, Kim Vettenranta, Mary Eapen |
Abstract |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
Canada | 1 | 33% |
United States | 1 | 33% |
Unknown | 1 | 33% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 1 | 33% |
Scientists | 1 | 33% |
Science communicators (journalists, bloggers, editors) | 1 | 33% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Finland | 1 | 3% |
United States | 1 | 3% |
Unknown | 38 | 95% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Other | 8 | 20% |
Researcher | 6 | 15% |
Student > Postgraduate | 4 | 10% |
Student > Doctoral Student | 2 | 5% |
Student > Bachelor | 2 | 5% |
Other | 10 | 25% |
Unknown | 8 | 20% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 24 | 60% |
Immunology and Microbiology | 2 | 5% |
Agricultural and Biological Sciences | 1 | 3% |
Engineering | 1 | 3% |
Unknown | 12 | 30% |