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[大血管血管炎合并骨髓增生异常综合征:一种罕见的关联]

[Large vessel vasculitis with myelodysplastic syndrome: A rare association].

作者信息

Galland J, Kawski H, Guichard J-F, Maurier F

机构信息

Service de médecine interne, hôpital Belle-Isle, hôpitaux privés de Metz, 2, rue Belle-Isle, 57000 Metz, France.

Service de médecine interne, hôpital Belle-Isle, hôpitaux privés de Metz, 2, rue Belle-Isle, 57000 Metz, France.

出版信息

Rev Med Interne. 2017 Jul;38(7):474-477. doi: 10.1016/j.revmed.2016.12.010. Epub 2017 Jan 13.

Abstract

INTRODUCTION

The vasculitis can be the consequence of malignancy: most often hematologic rather than solid tumors. The association between large vessels vasculitis and myelodysplastic syndrome is rare.

CASE REPORT

A 55-year-old man experienced asthenia, fever, polyarthritis and inflammatory syndrome. Haematological investigations found a type 2 refractory anemia with excess blasts (RAEB-2) with discovery of severe anemia (Hb: 7,8g/dl) and thrombopenia (platelets: 40,000/mm). Radiological examinations found thoracic aortitis and carotid vasculitis. Treatment in the form of steroids and azacitidine was instituted. The lack of control of both RAEB-2 and vasculitis was responsible for the death of the patient.

CONCLUSION

Myelodysplastic syndrome and large vessels vasculitis is a rare but serious association disease. The lack of efficiency of corticosteroids seems to be common. Prognosis depends on the haematological treatment effectiveness.

摘要

引言

血管炎可能是恶性肿瘤的后果:最常见的是血液系统肿瘤而非实体瘤。大血管血管炎与骨髓增生异常综合征之间的关联很少见。

病例报告

一名55岁男性出现乏力、发热、多关节炎和炎症综合征。血液学检查发现2型难治性贫血伴原始细胞增多(RAEB-2),伴有严重贫血(血红蛋白:7.8g/dl)和血小板减少(血小板:40,000/mm)。影像学检查发现胸主动脉炎和颈动脉血管炎。采用类固醇和阿扎胞苷进行治疗。RAEB-2和血管炎均未得到控制导致患者死亡。

结论

骨髓增生异常综合征和大血管血管炎是一种罕见但严重的关联疾病。皮质类固醇治疗效果不佳似乎很常见。预后取决于血液学治疗的有效性。

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