Treatment of children with Langerhans cell histiocytosis with 2-chlorodeoxyadenosine
Autor: | Patrick Kelly, Carlos Rodriguez-Galindo, Gerald G. Presbury, Michael Jeng, Martha Rieman, Winfred C. Wang |
---|---|
Rok vydání: | 2002 |
Předmět: |
medicine.medical_specialty
medicine.medical_treatment Purine analogue medicine.disease_cause Gastroenterology Disease-Free Survival Cohort Studies Pathogenesis Langerhans cell histiocytosis Recurrence Internal medicine Chlorodeoxyadenosine Humans Medicine Child Histiocyte Chemotherapy business.industry Remission Induction Infant Hematology Immune dysregulation medicine.disease Histiocytosis Langerhans-Cell Histiocytosis Treatment Outcome Child Preschool Immunology Cladribine business Immunosuppressive Agents |
Zdroj: | American Journal of Hematology. 69:179-184 |
ISSN: | 1096-8652 0361-8609 |
Popis: | 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/m(2)/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. |
Databáze: | OpenAIRE |
Externí odkaz: |