Mise en rémission par allogreffe de cellules souches hématopoïétiques d’une péri artérite noueuse associée à un syndrome myélodysplasique réfractaire à l’azacytidine

IF 0.7 4区医学 Q3 MEDICINE, GENERAL & INTERNAL

Revue De Medecine Interne Pub Date : 2024-08-01 DOI:10.1016/j.revmed.2024.06.002

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Abstract

Introduction

Periarteritis nodosa (PAN) is a vasculitis affecting medium-vessel and may be associated with myelodysplastic syndrome. This association needs a simultaneous treatment of the vascular and the hematological disease. However limited data are available on the benefit of hematological treatment, and in particular allogeneic stem cell transplantation, in this situation.

Case report

A 32-year-old patient with refractory periarteritis nodosa and simultaneous myelodysplastic syndrome, was treated with chemotherapy followed by hematopoietic stem cell allograft. The symptoms relating to PAN improved, allowing to decrease the dose of prednisone down to 5 mg/d. However, a hematological relapse occurred two months later leading to the patient's death.

Conclusion

Hematopoietic stem cell allograft may represent a therapeutic option in the management of severe or refractory autoimmune diseases when the hematological indication is retained.

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[同种异体造血干细胞移植诱导结节性动脉周围炎伴氮杂胞苷难治性骨髓增生异常综合征缓解]。

导言结节性动脉周围炎（PAN）是一种影响中血管的血管炎，可能与骨髓增生异常综合征有关。这种关联需要同时治疗血管疾病和血液疾病。然而，关于血液病治疗，特别是异体干细胞移植在这种情况下的益处，现有的数据非常有限：病例报告：一名 32 岁的患者患有难治性结节性动脉周围炎和骨髓增生异常综合征，在接受化疗后进行了造血干细胞异体移植。结节性动脉周围炎的相关症状有所改善，因此可以将泼尼松的剂量降至 5 毫克/天。然而，两个月后血液病复发，导致患者死亡：结论：造血干细胞异体移植可能是治疗严重或难治性自身免疫性疾病的一种治疗选择，但需保留血液学指征。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Revue De Medecine Interne 医学-医学：内科

CiteScore

0.70

自引率

11.10%

发文量

526

审稿时长

37 days

期刊介绍： Official journal of the SNFMI, La revue de medecine interne is indexed in the most prestigious databases. It is the most efficient French language journal available for internal medicine specialists who want to expand their knowledge and skills beyond their own discipline. It is also the main French language international medium for French research works. The journal publishes each month editorials, original articles, review articles, short communications, etc. These articles address the fundamental and innumerable facets of internal medicine, spanning all medical specialties. Manuscripts may be submitted in French or in English. La revue de medecine interne also includes additional issues publishing the proceedings of the two annual French meetings of internal medicine (June and December), as well as thematic issues.