Savaşan S, Warrier I, Ravindranath Y
Division of Hematology/Oncology, Children's Hospital of Michigan, Wayne State University, School of Medicine, Detroit 48201, USA.
Arch Dis Child. 1997 Sep;77(3):245-8. doi: 10.1136/adc.77.3.245.
Eleven patients (10 boys, one girl) with Evans' syndrome with a median follow up time of 8.0 years were evaluated retrospectively. Six patients had either persistent hepatosplenomegaly or generalised lymphadenopathy, or both. In five patients, an increase in lymph node and/or spleen size was observed during the exacerbations of cytopenias. Seven patients had quantitative serum immunoglobulin abnormalities at the time of presentation. There were associated systemic manifestations in nine patients. Various forms of treatment were used with mixed results. Four patients died from sepsis and haemorrhage; four had complete recovery--two after splenectomy. These findings show that Evans' syndrome is a heterogeneous disorder with significant morbidity and mortality. High incidence of quantitative serum immunoglobulin abnormalities, lymphoid hyperplasia, and associated systemic manifestations suggest that Evans' syndrome may represent a stage of a more broad spectrum, generalised immune dysregulation.
对11例埃文斯综合征患者（10例男孩，1例女孩）进行了回顾性评估，中位随访时间为8.0年。6例患者有持续性肝脾肿大或全身淋巴结肿大，或两者皆有。5例患者在血细胞减少症加重期间观察到淋巴结和/或脾脏大小增加。7例患者在就诊时存在血清免疫球蛋白定量异常。9例患者有相关的全身表现。采用了各种治疗方法，结果不一。4例患者死于败血症和出血；4例完全康复——2例在脾切除术后。这些发现表明，埃文斯综合征是一种异质性疾病，具有显著的发病率和死亡率。血清免疫球蛋白定量异常、淋巴组织增生及相关全身表现的高发生率提示，埃文斯综合征可能代表了更广泛的全身性免疫失调的一个阶段。
