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骨髓增生异常综合征中的血清阴性炎性关节炎。

Seronegative inflammatory arthritis in the myelodysplastic syndromes.

作者信息

George S W, Newman E D

机构信息

Department of Rheumatology, Geisinger Medical Center, Danville, PA 17822.

出版信息

Semin Arthritis Rheum. 1992 Jun;21(6):345-54. doi: 10.1016/0049-0172(92)90035-c.

DOI:10.1016/0049-0172(92)90035-c
PMID:1626280
Abstract

The myelodysplastic syndromes (MDS) are a group of therapeutically refractory anemias resulting from a clonal stem cell disorder often associated with cytogenetic abnormalities. Immunologic abnormalities and occasionally vasculitis have been reported although no series has characterized an associated arthritis. All cases of MDS diagnosed in 1990 by bone marrow biopsy and followed up at the authors' institution were reviewed. Of the 28 consecutive patients, 8 had acute seronegative inflammatory arthritis temporally related to the initial discovery of cytopenia. Five patients had a symmetric polyarthritis resolving only with use of steroids or upon evolution to leukemia, and 3 had episodes of oligoarthritis with systemic features including fever, pleuritis, pericarditis, and hemolytic anemia. Arthrocenteses in 2 cases did not show crystals or infection. Serological studies were nondiagnostic. The arthritis and systemic features responded to steroids in all 5 treated patients. Inflammatory arthritis appears to be common in MDS. Most compelling is the apparent bone marrow response to steroids in 2 cases, possibly identifying a treatable subgroup.

摘要

骨髓增生异常综合征(MDS)是一组由克隆性干细胞疾病引起的治疗难治性贫血,常伴有细胞遗传学异常。虽然尚无系列研究对相关关节炎进行特征描述,但已有免疫异常以及偶尔出现血管炎的报道。对1990年在作者所在机构通过骨髓活检诊断并随访的所有MDS病例进行了回顾。在这28例连续患者中,8例出现急性血清阴性炎症性关节炎,时间上与最初发现血细胞减少有关。5例患者患有对称性多关节炎,仅使用类固醇或演变为白血病后才缓解,3例出现寡关节炎发作,伴有包括发热、胸膜炎、心包炎和溶血性贫血在内的全身症状。2例关节穿刺未发现晶体或感染。血清学检查无诊断意义。所有5例接受治疗的患者的关节炎和全身症状对类固醇均有反应。炎症性关节炎在MDS中似乎很常见。最引人注目的是2例患者骨髓对类固醇有明显反应,这可能确定了一个可治疗的亚组。

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