The role of BMT in childhood histiocytoses

Caselli, Desireé

doi:10.1038/bmt.2008.46

Cited by 19 publications

(16 citation statements)

References 47 publications

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“…disease [16,17]. However, this procedure has a high mortality rate [16]. Before performing HSCT, it is important to control disease activity with minimum toxicity.…”

Section: Discussionmentioning

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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“…disease [16,17]. However, this procedure has a high mortality rate [16]. Before performing HSCT, it is important to control disease activity with minimum toxicity.…”

Section: Discussionmentioning

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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“…9 Allogeneic stem cell transplantation (SCT) has also been used because of its strong immunomodulatory effects for LCH. 10,11 This report describes the improved outcomes of 15 children with refractory LCH who underwent SCT in Japan.…”

Section: Introductionmentioning

confidence: 99%

Improved outcome of refractory Langerhans cell histiocytosis in children with hematopoietic stem cell transplantation in Japan

Kudo

Ohga

Morimoto

et al. 2009

Bone Marrow Transplant

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“…[12][13][14][15] In these patients, very intensive chemotherapy, similar to that used for acute myeloid leukemia, turned out to be useful, 19 whereas the role of hematopoietic stem cell transplantation remains uncertain. 20 But this is definitely the subset of patients for whom novel experimental therapies derived from improved knowledge of LCH pathogenesis appear warranted.…”

Section: Treatment Of Lchmentioning

confidence: 99%

Blood spotlight on Langerhans cell histiocytosis

Delprat

Aricò

2014

Blood

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Langerhans cell histiocytosis (LCH) is a rare disease affecting people of any age, with widely variable clinical manifestations and different outcomes. The precise chain of events driving lesional granuloma formation has remained elusive for many years. There is evidence for inherited predisposition to and derangement of apoptosis and inflammation in lesional dendritic cells. Recently somatic BRAFV600E mutation in myeloid precursor dendritic cells was associated with the more aggressive form of the disease, although the same mutation in a more differentiated dendritic cell might drive a less aggressive disease. Whether this picture convincingly put LCH in the field of myeloid neoplasm remains to be determined. Altogether, these findings suggest that future therapeutic strategy might incorporate a screening of this genetic mutation for high-risk patients potentially suitable for target therapy.

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The role of BMT in childhood histiocytoses

Cited by 19 publications

References 47 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

Improved outcome of refractory Langerhans cell histiocytosis in children with hematopoietic stem cell transplantation in Japan

Blood spotlight on Langerhans cell histiocytosis

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