Frayha R A, Shulman L E, Stevens M B
Acta Haematol. 1980;64(1):25-30. doi: 10.1159/000207206.
Hematological abnormalities in scleroderma indicate a specific complication of the disease itself, or an associated illness. Among 180 patients with scleroderma, anemia was detected in 25% and was attributed to chronic inflammatory disease (usually an overlap syndrome), bleeding mucosal telangiectases as part of the CREST syndrome, intestinal malabsorption, and microangiopathic hemolysis. Leukocytosis, present in 14%, was correlated with active myopathy and/or advanced visceral involvement while leukopenia was suggestive of a crossover with systemic lupus erythematosus. Thrombocytopenia was often a manifestation of microangiopathy, and thrombocytosis was associated with an arteritis or a tumor syndrome.
硬皮病中的血液学异常表明该疾病本身的特定并发症或相关疾病。在180例硬皮病患者中,25%检测到贫血,其原因是慢性炎症性疾病(通常是重叠综合征)、作为CREST综合征一部分的出血性黏膜毛细血管扩张、肠道吸收不良和微血管病性溶血。14%的患者存在白细胞增多,这与活动性肌病和/或晚期内脏受累相关,而白细胞减少提示与系统性红斑狼疮交叉。血小板减少常是微血管病的表现,而血小板增多与动脉炎或肿瘤综合征相关。