2-chlorodeoxyadenosine (2-CDA) for the treatment of refractory or recurrent Langerhans cell histiocytosis (LCH) in pediatric patients

Stine, Kimo C.; Saylors, Robert L.; Williams, Lawrence J.; Becton, David L.

doi:10.1002/(sici)1096-911x(199710)29:4<288::aid-mpo9>3.0.co;2-i

Cited by 49 publications

(16 citation statements)

References 15 publications

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“…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.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2010

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The aim of this study was to assess the outcome of treatment with 2-chlorodeoxyadenosine (2-CdA) in pediatric patients with Langerhans cell histiocytosis (LCH) in Japan. We retrospectively identified 17 pediatric LCH patients treated with 2-CdA. All patients were refractory or reactivated cases who had been initially treated according to the JLSG-02 protocol of the Japan LCH study group. At initiation of 2-CdA therapy, 4 patients had primary refractory multisystem (MS) disease with risk organ (RO) involvement (MS+), 9 patients had reactivated MS disease [5 MS+ and 4 without RO involvement (MS-)], and the remaining 4 patients had refractory/reactivated multifocal bone disease (MFB). Treatment with 2-CdA (4-9 mg/m(2)/day) was administered on 2-5 consecutive days and repeated every 3-4 weeks for a period that ranged from 2 to 12 months. Four primary refractory patients were treated with 2-CdA combined with high dose of cytarabine. In MS+ patients, response to treatment was observed in 5 of the 9 patients. In MS-/MFB patients, 5 of the 8 patients showed response to treatment. In the patients who were primary refractory or had reactivation during initial chemotherapy, 4 of 10 patients showed good response. On the other hand, in the patients having reactivation while off therapy, 6 of 7 patients showed good response. These findings suggest that 2-CdA is effective for reactivated LCH while off therapy.

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Section: Introductionmentioning

confidence: 99%

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

et al. 2010

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“…The thymic tumor suggested that the existing tumor in the right maxillary sinus represented a different type. The purine nucleoside analogue 2-chlorodeoxyadenosine (2-CDA) has been reported as being effective for infants with recurrent LCH (16). As this chemotherapy was not applied, the effectiveness of 2-CDA in the present case is unknown.…”

Section: Case Reportmentioning

confidence: 90%

Langerhans Cell Histiocytosis of the Maxillary Jaw in an 11-Month-Old Girl

Kitano¹,

Yoshida²,

Yamada

et al. 2012

Int J Oral-Med Sci

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Langerhans cell histiocytosis (LCH), a monoclonal disease of histiocytes, may involve several organ systems but rarely involves the maxillary sinus as a primary site. This report presents an extremely rare case of LCH of the maxillary jaw in an 11-month-old girl. An incisional biopsy for a maxillary lesion was performed, and a histopathological diagnosis of LCH was established. The histopathological examination revealed the proliferation of histiocytes with an infiltration of eosinophils. Histiocytes were positive for S-100 and CD68 (KPI). The patient subsequently underwent chemotherapy with the resolution of LCH in the maxillary jaw, and has continued to show partial response as of 1 year after chemotherapy.

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“…In children, the role of 2-CdA in the treatment of LCH is currently being de®ned. Stine et al recently reported three patients aged 2À18 years with multi-system LCH, refractory to conventional therapy, who had complete responses to 2-CdA 5À8 mg/m 2 given as a continuous infusion for 5À7 days [15]. The International Histiocyte Society gathered information regarding 15 patients (14 of them heavily pretreated) with multi-systemic LCH treated with 2-CdA.…”

Section: Discussionmentioning

confidence: 99%

Treatment of children with Langerhans cell histiocytosis with 2‐chlorodeoxyadenosine

et al. 2002

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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 signi®cant 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. Am.

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2-chlorodeoxyadenosine (2-CDA) for the treatment of refractory or recurrent Langerhans cell histiocytosis (LCH) in pediatric patients

Cited by 49 publications

References 15 publications

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

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

Langerhans Cell Histiocytosis of the Maxillary Jaw in an 11-Month-Old Girl

Treatment of children with Langerhans cell histiocytosis with 2‐chlorodeoxyadenosine

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