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      Localisations particulières de l'histiocytose langerhansienne chez l'enfant, scapula et pubis: à propos de deux cas Translated title: Specific locations of langerhans cell histiocytosis in children, scapula and pubis: report of two cases

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          L'histiocytose X ou histiocytose de Langerhans est une maladie de l'enfant et de l'adulte jeune. Dont l'incidence est estimée à 1 cas sur 200 000 par an. C'est une maladie au spectre clinique très divers, allant du simple granulome éosinophile à la forme grave multiviscérale avec dysfonctionnement d'organe. Les auteurs rapportent deux observations concernant deux localisations assez rares de cette maladie, au niveau du pubis chez le premier enfant, et au niveau de la scapula chez le deuxième. Chez nos deux malades la localisation était focale, et l’évolution était favorable. A travers ces deux observations, nous allons essayer de décrire les différents aspects cliniques et radiologiques et discuter a travers une revue de littérature les démarches diagnostiques et thérapeutiques de cette maladie rare.

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          Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome.

          The objective of this descriptive analysis of a large cohort of patients with Langerhans cell histiocytosis (LCH) was to add to the understanding of the natural history, management, and outcome of this disease. Three hundred fourteen Mayo Clinic patients with histologically proven LCH were categorized into those patients with multisystem disease and those patients with single system disease. Clinical features, treatment, and outcome were determined from the case history notes and tumor registry correspondence. Treatment included chemotherapy, radiotherapy, and surgical excision. The end points were disease free survival, active disease, or death. The median time of follow-up was 4 years (range, 1 month to 47.5 years). The age of the patients ranged from 2 months to 83 years. Of the 314 patients, there were 28 deaths. Multisystemic LCH was found in 96 patients, 25 of whom had continuing active disease after treatment. Isolated bone LCH lesions were observed in 114 of the 314 patients, 111 of whom (97%) achieved disease free survival after treatment. The most common sites of osseous LCH were the skull and proximal femur. Of the 87 patients with isolated pulmonary involvement, only 3 were nonsmokers. After treatment with corticosteroids (+/- cyclophosphamide or busulphan), 74 patients achieved disease free survival, but 10 patients died. Pituitary-thalamic axis LCH, characterized by diabetes insipidus, was found in 44 patients. After treatment, 30 of these patients had disease free survival, but all required long term hormone replacement with desmopressin acetate. Lymph node involvement was found in 21 patients, and mucocutaneous involvement was found in 77 patients. Patients with isolated bone LCH lesions have the best prognosis compared with patients with LCH involvement of other systems. By contrast, 20% of patients with multisystem involvement have a progressive disease course despite treatment. The identification of prognostic indicators to facilitate appropriate treatment and long term follow-up surveillance is recommended.
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            Langerhans' cell histiocytosis (histiocytosis X) of bone. A clinicopathologic analysis of 263 pediatric and adult cases.

            Langerhans' cell histiocytosis (LCH) of bone is a disorder of histiocytic proliferation with variable and often unpredictable behavior. The authors evaluated the clinical and pathologic features of 263 patients (172 children, 91 adults) with biopsy-proven LCH examined during an 80-year period at the Mayo Clinic. Only patients with bone involvement pathologically and/or radiographically were included in the study. Clinical follow-up was available for 245 patients and ranged from 3 months to 50 years (mean, 12 years; median, 10 years). Chi-square tests were used to determine associations between age, gender, extent of osseous involvement, visceral disease, and pathologic features. Survival analyses were performed by univariate and multivariate Cox regression methods. Age at presentation ranged from 2 months to 71 years with a clear predominance in children. The most common presenting complaint was pain, often worse at night. The skull was the most frequent osseous site in children and adults. Diabetes insipidus was documented in 40 patients. Forty-four children developed skeletal recurrence and/or new bone lesions, 19 of whom had diabetes insipidus. Fourteen children and 3 adults died either directly or indirectly from LCH. One adult patient developed systemic amyloidosis. All but two of these pediatric patients were 3 years of age or younger at presentation. All children with hepatosplenomegaly (7 patients) and/or thrombocytopenia (9 patients) died. Nine of the 14 children who died presented initially with three or more bone lesions. The clinical behavior of LCH of bone is often unpredictable; however, young age at diagnosis, hepatosplenomegaly, thrombocytopenia, and polyostotic (> or = 3 bones involved) disease are associated with a poor prognosis (P < 0.005). Recrudescence in children, but not in adults, strongly correlates with the presence of diabetes insipidus (P < 0.0005).
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              Percutaneous techniques for the diagnosis and treatment of localized Langerhans-cell histiocytosis (eosinophilic granuloma of bone).

              We retrospectively studied the outcome of percutaneous needle biopsy and intralesional injection of a corticosteroid (methylprednisolone) in thirty-nine patients who had localized Langerhans-cell histiocytosis (eosinophilic granuloma of bone). All thirty-nine patients had a solitary symptomatic lesion at presentation; a second lesion developed in two patients, two and four months after the first lesion was diagnosed. Therefore, there were forty-one lesions in thirty-nine patients. Fine-needle aspiration with or without core-needle biopsy was performed for all forty-one lesions, and the diagnosis of Langerhans-cell histiocytosis was established for thirty-seven (90 per cent). A corticosteroid was injected into thirty-five lesions. Twenty-nine received the injection at the time of the fine-needle aspiration on the basis of the cytological findings in the aspirate. Six patients who had a solitary lesion had a two-stage procedure because the injection was delayed until the diagnosis was confirmed with histological evaluation of specimens obtained by core-needle biopsy. Thirty-four (97 per cent) of the thirty-five lesions healed. The clinical symptoms associated with thirty-one lesions resolved within two weeks after a single injection of the corticosteroid. There were no complications associated with either the biopsy or the injection. At a median of ninety months (range, twenty-four to 199 months), no patient had recurrence of symptoms or of radiographic evidence of the lesion. All patients who had been managed with an intralesional injection of the corticosteroid had full range of motion of the affected extremity and had resumed unlimited activities. Although the mechanism of action of intralesional injection of a corticosteroid has not been defined, use of percutaneous needle biopsy to diagnose localized Langerhans-cell histiocytosis and treatment with intralesional administration of methylprednisolone relieved pain expeditiously, enabled the patient to avoid an operative procedure, and resulted in osseous healing. The specific role of corticosteroid therapy remains to be determined by prospective, randomized studies.

                Author and article information

                Pan Afr Med J
                Pan Afr Med J
                The Pan African Medical Journal
                The African Field Epidemiology Network
                25 August 2014
                : 18
                [1 ]Service de Chirurgie Traumato-orthopédie, Pédiatrique, CHU Hassan II, Faculté de Médecine et de Pharmacie, Université sidi Mohammed ben Abdullah, Fès
                Author notes
                [& ]Corresponding author: Atarraf Karima, Service de Chirurgie Traumato-orthopédie, Pédiatrique CHU Hassan II, Fès, Maroc
                © Atarraf Karima et al.

                The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Case Report


                histiocytose x, scapula, ischion, enfant, histiocytosis x, child


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