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Résumé: Le signe du rein chevelu est classiquement reconnu comme pathognomonique d'une maladie d'Erdheim-Chester, histiocytose non langheransienne rare. Observation clinique : un patient de 72 ans, suivi pour une leucémie myélomonocytaire chronique (LMMC) à JAK2 (pour Janus kinase 2) muté, a présenté une insuffisance rénale aiguë, faisant découvrir au scanner un aspect de rein chevelu. La biopsie rénale a révélé un foyer d'hématopoïèse extramédullaire de la LMMC. Nous avons observé une amélioration de la fonction rénale sous ruxolitinib. Ceci n'a jamais été décrit jusqu'alors. Conclusion : si l'aspect de rein chevelu est considéré comme pathognomonique d'une maladie d'Erdheim-Chester, un foyer d'hématopoïèse extramédullaire – comme dans la LMMC, dans ce cas – peut être évoqué. Il s'agit du premier cas décrit de réponse thérapeutique favorable au ruxolitinib dans le cadre d'une infiltration rénale de la LMMC. Hairy kidney's sign is usualy discribe like a pathognomonic sign of Erdheim-Chester disease, rare, non-Langerhans cell histiocytosis. Case report: A 72-years-old men, kown for chronic myelomonocytic leukemia (CMML) JAK2 muted, presented an acute kidney failure, revealing un hairy kidney's sign at computed tomography. Kidney biopsy find an extramedullary hematopoeisis of CMML. We observed an improvement of the renal function with ruxolitinib. It was never been described until then. Conclusion: Hairy kidney's sign is usualy discribe like a pathognomonic sign of Erdheim-Chester disease, however, an extramedullary hematopoeisis of CMML, in this case, could be considered. In addition, this is the first reported case of a favorable therapeutic response to ruxolitinib in kidney infiltration of CMML. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To assess the benefits and harms to initiate corticosteroids with intravenous methylprednisolone at a conventional dose (1 mg/kg/d) to treat adults with immune thrombocytopenia (ITP). Methods: Population stemmed from the prospective multicenter CARMEN registry and included newly diagnosed hospitalized ITP adults with platelet counts<30 × 109/L. We compared the patients treated with conventional‐dose methylprednisolone (CDMP) before continuing with oral prednisone to patients treated with just conventional‐dose oral prednisone (CDOP). The primary outcome was the time until response. Secondary outcomes were time until complete response, response rate, complete response rate, duration of hospital stay, and occurrence of adverse drug reactions. Analyzes were adjusted for propensity score and for exposure to intravenous immunoglobulin. Results: Among the included 87 patients, the median time to response was 3 days in the CDMP group vs 4 in the CDOP group (adjusted hazard ratio [aHR]: 1.35; 95%CI: 0.76‐2.41). The CDMP group had an earlier complete response (aHR: 2.29; 95%CI: 1.20‐4.36). There was no difference between the groups regarding other secondary outcomes. Conclusions: Initiating methylprednisolone at a conventional dose provided no significant benefit compared to giving oral prednisone only to adults with ITP. [ABSTRACT FROM AUTHOR]