Long‐term follow‐up of children with risk organ‐negative Langerhans cell histiocytosis after 2‐chlorodeoxyadenosine treatment

Barkaoui, Mohamed‐Aziz; Queheille, Emma; Aladjidi, Nathalie; Plat, Geneviève; Jeziorski, Éric; Moshous, Despina; Lambilliotte, Anne; Kébaïli, Kamila; Pacquement, Hélène; Leverger, G; Mansuy, L; Entz‐Werlé, Natacha; Bodet, Damien; Schneider, Pascale; Pagnier, Anne; Lutun, Anne; Gillibert-Yvert, Marion; Millot, Frédéric; Toutain, F.; Réguerre, Yves; Thomas, Caroline; Tazi, A.; Émile, Jean-François; Donadieu, Jean; Héritier, Sébastien

doi:10.1111/bjh.16944

Cited by 15 publications

(15 citation statements)

References 38 publications

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“…By contrast, chemotherapies have been found to be more effective for neurologic LCH, including vinblastine in one retrospective series of 20 pediatric patients [ 25 ] in which 15 (75%) had CR or PR. Cladribine was observed to lead to response without relapse in 17/17 pediatric patients with CNS LCH in one French series [ 26 ], as well as 12/12 responses in a series of mixed adults and children with CNS LCH in another series [ 27 ]. Finally, this review provides very little data regarding radiotherapy only for neurologic RDD with 4 instances as first-line treatment (1 PR, 2 SD, 1 POD) and 10 as second-line treatment (1 CR, 4 PR, 1 SD, 2 POD, 2 unknown), but it would seem that further study of this treatment may be merited.…”

Section: Discussionmentioning

confidence: 99%

Rosai–Dorfman–Destombes disease of the nervous system: a systematic literature review

Nasany

Reiner

Francis

et al. 2022

Orphanet J Rare Dis

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Background Rosai–Dorfman–Destombes disease (RDD) is a rare histiocytic disorder with heterogeneous clinical manifestations and rare neurologic involvement. The existing clinical literature about neurologic RDD has yet to be critically examined. Methods We performed a four-database English-language systematic literature search for cases of RDD neurohistiocytosis, excluding secondary literature. Individual patient data for neurologic symptoms, disease sites, treatments, and responses were captured. Responses to first-line and second-line surgical interventions, post-surgical radiotherapy, and systemic therapies were analyzed. Results Among 4769 articles yielded by literature search, 154 articles were fully reviewed, containing data on 224 patients with neurologic RDD. 128 (83.1%) articles were single case reports. 149 (66.5%) patients were male, 74 (33.5%) female, with a median age of 37.6 years (range 2–79). Presenting neurologic symptoms included headache (45.1%), focal neurological deficits (32.6%), visual symptoms (32.1%), and seizures (24.6%). RDD involvement was multifocal in 32 (14.3%) cases. First-line treatment involved resection in 200 (89.6%) patients, with subsequent progression in 52 (26%), including 41 (78.8%) with unifocal disease. No difference was observed in progression-free survival comparing post-operative radiotherapy to no radiotherapy following partial resection. Chemotherapy given alone as first-line treatment led to complete or partial response in 3/7(43%) patients. Second-line treatments led to complete or partial response in 18/37(37.5%) patients. Mutational data were reported on 10 patients (4.46%). Conclusions This review highlights the limited published data about neurologic RDD, which presents with varied symptomatology and outcome. Further study is needed about its mutational landscape, and more effective therapies are needed for recurrent and refractory disease.

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Section: Discussionmentioning

confidence: 99%

Rosai–Dorfman–Destombes disease of the nervous system: a systematic literature review

Nasany

Reiner

Francis

et al. 2022

Orphanet J Rare Dis

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“…In subsequent courses, we administrated 2-CdA monotherapy, which is effective and safe for the low-risk LCH patient, to avoid adverse effects, such as myelosuppression and mucositis. 8 To the best of our knowledge, there were only 6 cases of extra-JXG and sJXG with CNS, which were treated with 2-CdA reported since 2005. 9-13 Four cases were treated with 2-CdA monotherapy, 1 with 2-CdA and dexamethasone, and 1 with 2-CdA and cytarabine.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, we decided to use the combination therapy of 2-CdA and cytarabine as first salvage chemotherapy to control his progressive condition. In subsequent courses, we administrated 2-CdA monotherapy, which is effective and safe for the low-risk LCH patient, to avoid adverse effects, such as myelosuppression and mucositis 8…”

Section: Discussionmentioning

confidence: 99%

Refractory Extracutaneous Juvenile Xanthogranuloma With Multiple Intracranial Nodular Lesions Successfully Treated With 2-Chlorodeoxyadenosine

Matsukawa

Sakamoto

Shibata

2021

Journal of Pediatric Hematology/Oncology

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Juvenile xanthogranulomatosis (JXG) is a rare histiocytic disease that is usually limited to the skin, but some JXG cases involve other organs. JXG involving the central nervous system (CNS) is rare and its treatment is inadequate. The optimum treatment for refractory JXG involving the CNS remains unknown. We report here a case of refractory pediatric extracutaneous JXG (extra-JXG) involving the CNS with multiple intracranial masses treated with 2-chlorodeoxyadenosine resulting in achievement of long-term complete remission. 2-Chlorodeoxyadenosine, with favorable CNS penetration in the cerebrospinal fluid, is apparently an effective treatment for extra-JXG and systemic JXG (sJXG) with CNS involvement.

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“…Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm characterized by clonal proliferation of Langerhans cells in various organs, including skin, bones, lymph nodes, soft tissue, lung and liver. LCH can occur in all age groups, but the first onset of this disorder tends to occur in children (1,2). Conventional imaging modalities such as X-ray, CT and MRI play a key role in LCH not only in diagnosis and evaluating the extent of involvement but also in guiding biopsy and follow-up (2-5), and 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography/computed tomography (PET/CT) has also been increasingly used (4,6,7).…”

Section: Introductionmentioning

confidence: 99%

Role of 18F-FDG PET/CT in the diagnosis and management of patients with Langerhans cell histiocytosis

Luo¹,

Lu²,

Qi³

et al. 2022

Quant Imaging Med Surg

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Background: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm that can involve multisystem organs. Positron emission tomography/computed tomography (PET/CT) has been widely used in tumor staging and efficacy evaluation. However, at present, there are few 18 F-fluorodeoxyglucose (FDG) PET/CT studies on LCH. This study aimed to explore the possible role of 18 F-FDG PET/CT in the diagnosis and management of patients with LCH.Methods: 18 F-FDG PET/CT images of 22 Chinese patients diagnosed with LCH on biopsy or surgery histopathology between January 2011 and December 2020 were retrospectively analyzed. The incidence of LCH in each system was assessed by a PET/CT scan. The imaging characteristics were analyzed semiquantitatively and qualitatively. The discrepancies between PET/CT and conventional imaging modalities were recorded. Evaluations of curative effect according to RECIST1.1 and PERCIST1.0 were compared using Fisher's exact chi-squared test, and P values <0.05 were considered significant.Results: Eight (36.4%) of the 22 patients presented with single system involvement (4 isolated site involvement, 4 multiple site involvement), and 14 (63.6%) presented with multiple system involvement.Twenty-one (95.5%) patients had hypermetabolic lesions. Musculoskeletal, lymphatic, respiratory, liver, skinsoft tissue and thyroid involvement were seen in 14 (63.6%), 13 (59.1%), 5 (22.7%), 4 (18.2%), 5 (22.7%) and 1 (4.5%) patient, respectively. Cranial and facial bones were the most common sites of musculoskeletal involvement. Ten patients underwent PET/CT follow-up, and there was no significant difference in TR evaluations according to RECIST1.1 and PERCIST1.0. However, among the complete remission cases assessed by RECIST1.1, three were partial metabolic responses assessed by PERCIST1.0, while among the partial response cases assessed by RECIST1.1, one was metabolic progressive disease assessed by PERCIST1.0.Conclusions: 18 F-FDG PET/CT is an imaging modality option for the diagnosis and assessment of the TR of LCH.

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Long‐term follow‐up of children with risk organ‐negative Langerhans cell histiocytosis after 2‐chlorodeoxyadenosine treatment

Cited by 15 publications

References 38 publications

Rosai–Dorfman–Destombes disease of the nervous system: a systematic literature review

Rosai–Dorfman–Destombes disease of the nervous system: a systematic literature review

Refractory Extracutaneous Juvenile Xanthogranuloma With Multiple Intracranial Nodular Lesions Successfully Treated With 2-Chlorodeoxyadenosine

Role of 18F-FDG PET/CT in the diagnosis and management of patients with Langerhans cell histiocytosis

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