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Clinical analysis of primary Sjögren’s syndrome complicating anemia

机译:原发性干燥综合征并发贫血的临床分析

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This is a cross-sectional study to assess the prevalence and causes of anemia in the primary Sjögren’s syndrome (pSS). One hundred and thirty-two consecutive patients with pSS were enrolled into the study. Standard hematological and immunological tests and examination of bone marrow were performed. Anemia occurred in 45 (34.1%) patients. The causes of anemia included anemia of chronic disease (69%), autoimmune hemolytic anemia (AIHA, 18%), iron deficiency anemia (9%) and other causes (4%), of which AIHA caused the most severe anemia. The prevalence of ANA, anti-Ro/SSA, and anti-La/SSB was much higher in patients with anemia than those without anemia. Anticardiolipin antibodies were most commonly detected in AIHA; the prevalence of IgG and hypocomplementemia in AIHA was much higher in patients without anemia. Abnormal bone marrow changes were observed in two cases with anemia, one with morphological changes in the myeloid, megakaryocytic, and erythroid lineages and one with hypocellularity in the erythroid lineage. Therefore, pSS patients with anemia may be associated with destruction of peripheral mature blood cells, impaired red cells production, and hematopoietic abnormalities due to an immune mechanism, although the concrete pathogenesis is still unclear.
机译:这是一项横断面研究,旨在评估原发性干燥综合征(pSS)的贫血患病率和原因。连续132例pSS患者被纳入研究。进行标准血液学和免疫学检查以及骨髓检查。 45名(34.1%)患者发生贫血。贫血的原因包括慢性疾病性贫血(69%),自身免疫溶血性贫血(AIHA,18%),铁缺乏性贫血(9%)和其他原因(4%),其中AIHA引起最严重的贫血。贫血患者中ANA,抗Ro / SSA和抗La / SSB的患病率比无贫血患者高得多。抗心磷脂抗体在AIHA中最常见;没有贫血的患者中AIHA中IgG和低补体血症的患病率要高得多。在2例贫血患者中观察到了异常的骨髓变化,其中1例发生了髓样,巨核细胞和红系谱系的形态变化,另一例发生了红系谱系的细胞减少。因此,尽管尚不清楚具体的发病机制,但由于免疫机制,患有贫血的pSS患者可能与周围成熟血细胞破坏,红细胞生成受损以及造血异常有关。

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