Treatment of children with Langerhans cell histiocytosis with 2‐chlorodeoxyadenosine

Langerhans cell histiocytosis (LCH) is a disorder characterized by proliferation of activated Langerhans cells. Immune dysregulation is believed to be part of the pathogenesis. Although current therapies are very effective at inducing remission, multiple recurrences and long‐term sequelae are common...

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Bibliographic Details
Published in:American journal of hematology 2002-03, Vol.69 (3), p.179-184
Main Authors: Rodriguez‐Galindo, Carlos, Kelly, Patrick, Jeng, Michael, Presbury, Gerald G., Rieman, Martha, Wang, Winfred
Format: Article
Language:English
Subjects:
2‐chlorodeoxyadenosine
Antineoplastic agents
Biological and medical sciences
Chemotherapy
Child
Child, Preschool
children
Cladribine - administration & dosage
Cladribine - standards
Cohort Studies
Disease-Free Survival
Histiocytosis, Langerhans-Cell - drug therapy
Histiocytosis, Langerhans-Cell - pathology
Humans
Immunosuppressive Agents - administration & dosage
Immunosuppressive Agents - standards
Infant
Langerhans cell histiocytosis
Medical sciences
Pharmacology. Drug treatments
Recurrence
Remission Induction
Treatment Outcome
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Summary:Langerhans cell histiocytosis (LCH) is a disorder characterized by proliferation of activated Langerhans cells. Immune dysregulation is believed to be part of the pathogenesis. Although current therapies are very effective at inducing remission, multiple recurrences and long‐term sequelae are common for patients with low‐risk disease, and a significant proportion of young patients die of their disease. More effective therapies based on the pathogenesis of LCH are needed. We investigated the use of 2‐chloro‐deoxyadenosine (2‐CdA), a purine analogue with an antiproliferative effect on histiocytes and lymphocytes, in patients with recurrent or high‐risk LCH. Six patients with recurrent LCH received 2‐CdA (5–7 mg/m2/day for 5 days, repeated every 21–28 days). All patients achieved remission. With a median follow‐up of 15 months (range, 3–25 months), 5 patients remain in remission. A patient with multisystem disease who recurred after 13 months, achieved a second remission with 2‐CdA. Hematologic toxicity was minimal, and no infectious complications were documented. 2‐CdA is among the most effective drugs for the treatment of LCH, and this is probably due to both its anti‐proliferative and immunomodulatory effects. 2‐CdA needs to be considered for the treatment of recurrent LCH. However, its incorporation into front‐line treatment of patients with multi‐system LCH needs further study. Am. J. Hematol. 69:179–184, 2002. © 2002 Wiley‐Liss, Inc.
ISSN:0361-8609
1096-8652
DOI:10.1002/ajh.10053