1. Multi-centre pilot study of 2-chlorodeoxyadenosine and cytosine arabinoside combined chemotherapy in refractory Langerhans cell histiocytosis with haematological dysfunction
- Author
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Y. Perel, Christopher P. Thomas, Jean Donadieu, J. Landman Parker, V. Minard Colin, Christiane Vermylen, Pascal Chastagner, Yves Bertrand, Marie Ouache, Frédéric Bernard, and Martine Munzer
- Subjects
Male ,Cancer Research ,medicine.medical_specialty ,medicine.drug_class ,Pilot Projects ,Gastroenterology ,Antimetabolite ,Langerhans cell histiocytosis ,Refractory ,Internal medicine ,Antineoplastic Combined Chemotherapy Protocols ,medicine ,Chlorodeoxyadenosine ,Humans ,Infusions, Intravenous ,Cladribine ,business.industry ,Cytarabine ,Infant ,Combination chemotherapy ,medicine.disease ,Hematologic Diseases ,Survival Analysis ,Surgery ,Transplantation ,Histiocytosis, Langerhans-Cell ,Oncology ,Chronic Disease ,Drug Therapy, Combination ,Female ,business ,medicine.drug - Abstract
The aim of this study was to assess the efficacy and adverse effects of 2-chlorodeoxyadenosine (2-CdA) and cytosine arabinoside (Ara-C) in children with refractory Langerhans cell histiocytosis (LCH) and haematopoietic dysfunction. Ten patients, with a median age at diagnosis of 0.5 years, were enrolled in this study. Treatment comprised at least two courses of Ara-C (1000mg/m 2 /d) and 2-CdA (9mg/m 2 /d) administered for 5d every 4 weeks; subsequent median follow-up was 2.8 years (range 0.03–6.4 years). Among the 7 patients who received at least two courses of therapy, disease activity decreased in 6 patients, and control of disease was achieved in all patients after a median delay of 5.5 months. All patients suffered World Health Organisation (WHO) grade 4 haematological toxicity. Two septic deaths occurred shortly after administration of the first course of 2-CdA/Ara-C; a third patient was withdrawn from the trial after the first course and subsequently died following haematopoietic stem cell transplantation. This series is small, but we conclude that 2-CdA and Ara-C combined chemotherapy probably has major activity in childhood refractory Langerhans cell histiocytosis.
- Published
- 2005
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