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骨髓增生异常综合征与自身免疫:一例骨髓增生异常综合征不寻常表现的病例报告

Myelodysplastic syndrome and autoimmunity: a case report of an unusual presentation of myelodysplastic syndrome.

作者信息

Merrill Andrea L, Smith Hedy

机构信息

Tufts University School of Medicine, Boston, MA 02111, USA.

出版信息

Case Rep Hematol. 2011;2011:560106. doi: 10.1155/2011/560106. Epub 2011 Sep 22.

Abstract

Myelodysplastic syndrome (MDS) commonly presents asymptomatically or with symptomatic cytopenias. However, autoimmune phenomena in association with MDS have been well described in several case reports and case series. Typically, these autoimmune phenomena take the form of vasculitides, arthritis, connective tissue diseases, pulmonary infiltrates, or polymyalgia rheumatica. We present the case of a woman with MDS (karyotype 46,XX,+1,der(1;7)(q10;p10)[20], that evolved with an additional trisomy 8 clone) and a novel spectrum of autoimmune diseases including acute fibrinous and organizing pneumonia (AFOP) and lacrimal gland pseudotumor.

摘要

骨髓增生异常综合征(MDS)通常无症状表现或伴有症状性血细胞减少。然而,在一些病例报告和病例系列中已对与MDS相关的自身免疫现象进行了充分描述。典型地,这些自身免疫现象表现为血管炎、关节炎、结缔组织病、肺部浸润或风湿性多肌痛。我们报告了一例患有MDS(核型为46,XX,+1,der(1;7)(q10;p10)[20],并伴有额外的8号染色体三体克隆)的女性病例,该病例还出现了包括急性纤维素性机化性肺炎(AFOP)和泪腺假瘤在内的一系列新的自身免疫性疾病。

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