Treatment of recurrent Langerhans cell histiocytosis in children with 2-chlorodeoxyadenosine

Abstract

The objective of this study was to evaluate the efficacy of 2-chlorodeoxyadenosine (2-CdA), a purine nucleoside analog, in treating recurrent Langerhans cell histiocytosis (LCH) in children. This study retrospectively analysed the clinical records of 13 patients who were seen in the department for recurrent LCH. These patients were treated consecutively with 2-CdA chemotherapy between July 1997 and May 2005. Median age at diagnosis was 4 years 7 months and median pre-treatment duration of disease was 16.4 months. Four children received 0.1 mg kg-1 per day for 7 days and nine patients 5 mg m-2 per day for 5 days, repeated every 21 days. The maximum number of courses of 2-CdA per patient was limited to six. Seventy-six courses of 2-CdA were administered without difficulty. All 13 patients (100%) had a clinical response documented by radiographic investigation. Nine patients did not require additional therapy and remain in complete remission (CR). Four remaining children are currently disease-free after receiving other therapy as irradiation (two cases) or maintenance chemotherapy (vinblastine, prednisone and 6-mercaptopurine) (one case) or chemotherapy (vinblastine) + irradiation (one child) ( Table I). Hematologic toxicity was minimal and no infectious complications were documented. Median follow-up after initiation of 2-CdA treatment was 4 years 3 months (range 7 months - 8 years 2 months). This experience confirms the reported efficacy of 2-CdA in the treatment of LCH. However, further studies are needed to determine the role of this agent in high-risk patient who did not achieve complete remission after 2-CdA administration.

Outcome of pediatric patients with Langerhans cell histiocytosis treated with 2 chlorodeoxyadenosine: a nationwide survey in Japan

“…Several recent reports have shown that 2-chlorodeoxyadenosine (2-CdA), which is a purine substrate analog, is an effective treatment for refractory or reactivated childhood MS LCH [3,4]. In adult LCH patients, 2-CdA has been used as a first-line therapy [5], but its efficacy in childhood LCH is unclear.…”

Immunohistochemical evaluation of cell proliferation and apoptosis markers in ovarian surface epithelial cells of cladribine-treated rats

“…However, currently, PNA administration has become a useful tool in treating young persons and even children. Indeed, positive effects of cladribine in the treatment of chronic lymphocytic leukemia have been described (Chow et al 2000), as its efficacy in treating NHL [Chow et al 2000], mantle cell lymphoma (Inwards et al 2008), AML (Wierzbowska et al 2008), myelodysplastic syndromes (Laurencet et al 1997), chronic myeloid leukemia (Martinelli et al 1996), Waldenström macroglobulinemia (Rourke et al 2010), histiocytosis, also in children (Mottl et al 2006), astrocytomas (Ceruti et al 2000, Ceruti et al 2003), multiple sclerosis (Hartung 2009; Leist and Weissert 2011; Vosoughi and Freedman 2010), mast cell disease (Aichberger et al 2008), psoriasis, including psoriatic arthritis (Eibschutz et al 1995), systemic lupus erythematosus (Bernknopf et al 2011), angioedema, and anaphylaxis (Dutta et al 2002). …”

Langerhans cell histiocytosis of vulva in adolescent

“…Isolated LCH as bone and genital lesions have a tendency to spontaneous regression, and radiotherapy should not be a front line treatment, mainly for adolescents (20,26) . Some experiences confirmed efficacy of 2-CdA in the treatment of LCH (27)(28)(29)30) . The primary systemic chemotherapy with VBL and PDN (LCH II-LR protocol) in our patient showed only an 8-month remission followed by a local recurrence.…”