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与骨髓增生异常综合征和内脏利什曼病相关的新月体性肾小球肾炎。

Crescentic glomerulonephritis associated with myelodysplatic syndrome and visceral leishmaniasis.

作者信息

Decaestecker Antoine, Daniel Adrien, Malbranque Zoé, Wayolle Nicolas, Ydee Amandine

机构信息

Department of Nephrology, Centre Hospitalier de Dunkerque, 130 Av. Louis Herbeaux, 59240, Dunkirk, France.

Department of Hematology, Centre Hospitalier de Dunkerque, Dunkirk, France.

出版信息

J Nephrol. 2025 Jun 19. doi: 10.1007/s40620-025-02329-y.

Abstract

Membranoproliferative glomerulonephritis (MPGN) can be secondary to infections, autoimmune diseases, or hematological disorders. MPGN is uncommon and has a poor renal prognosis. We report the case of a patient with a myelodysplastic syndrome, presenting with autoimmune inflammatory manifestations and rapidly progressing kidney failure. The kidney biopsy revealed MPGN with extracapillary proliferation. The patient's condition rapidly improved under corticosteroid therapy, with normalization of the kidney function. However, during corticosteroid treatment, the patient developed severe visceral leishmaniasis with cutaneous, splenic, and osteo-medullary involvement. Upon review, we found that parasites were already present in minimal quantities in the bone marrow biopsy performed three months prior to corticosteroid treatment. We cannot conclusively determine whether the kidney lesions are linked to the autoimmune inflammatory manifestations of the myelodysplastic syndrome or to leishmaniasis. This case may be noteworthy due to the combination of two diseases that could be responsible for the histological lesions, and emphasizes the importance of a thorough evaluation in the case of MPGN.

摘要

膜增生性肾小球肾炎(MPGN)可继发于感染、自身免疫性疾病或血液系统疾病。MPGN并不常见,肾脏预后较差。我们报告一例骨髓增生异常综合征患者,伴有自身免疫性炎症表现并迅速进展为肾衰竭。肾脏活检显示为伴有毛细血管外增生的MPGN。患者在糖皮质激素治疗下病情迅速改善,肾功能恢复正常。然而,在糖皮质激素治疗期间,患者发生了严重的内脏利什曼病,累及皮肤、脾脏和骨髓。经复查,我们发现寄生虫在糖皮质激素治疗前三个月进行的骨髓活检中就已少量存在。我们无法最终确定肾脏病变是与骨髓增生异常综合征的自身免疫性炎症表现有关,还是与利什曼病有关。该病例可能值得关注,因为两种疾病的合并可能导致组织学病变,并强调了MPGN病例进行全面评估的重要性。

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