Gentile T C, Loughran T P
Department of Medicine, State University of New York at Syracuse, USA.
Leuk Lymphoma. 1996 Oct;23(3-4):405-8. doi: 10.3109/10428199609054846.
A 24 year old female with a 4 year history of anemia and absolute lymphocytosis was evaluated and found to have T cell large granular lymphocyte (T-LGL) leukemia associated with autoimmune hemolytic anemia, neutropenia, mild thrombocytopenia and splenomegaly. In an effort to ameliorate her symptomatic cytopenias, she was treated with prednisone and subsequently methotrexate without success. In February 1993, she underwent splenectomy for symptomatic anemia. Splenectomy resulted in an increased hemoglobin concentration to normal levels, resolution of all laboratory evidence of hemolysis, and disappearance of thrombocytopenia. This response has been durable despite persistence of the abnormal LGL clone. We suggest that splenectomy may be an effective treatment for autoimmune hemolytic anemia and/or thrombocytopenia often associated with T-LGL leukemia. As this disease often exhibits a chronic clinical course with morbidity resulting from consequences of resultant cytopenias rather than visceral involvement with leukemic LGL, effective treatment of cytopenias despite persistence of the abnormal LGL clone is beneficial.
一名24岁女性,有4年贫血和绝对淋巴细胞增多病史,经评估发现患有与自身免疫性溶血性贫血、中性粒细胞减少、轻度血小板减少和脾肿大相关的T细胞大颗粒淋巴细胞(T-LGL)白血病。为改善其症状性血细胞减少,她接受了泼尼松治疗,随后使用甲氨蝶呤,但均未成功。1993年2月,她因症状性贫血接受了脾切除术。脾切除术后血红蛋白浓度升至正常水平,所有溶血实验室证据均消失,血小板减少症也消失。尽管异常LGL克隆持续存在,但这种反应一直持续。我们认为脾切除术可能是治疗常与T-LGL白血病相关的自身免疫性溶血性贫血和/或血小板减少症的有效方法。由于这种疾病通常表现为慢性临床病程,其发病是由血细胞减少的后果而非白血病LGL的内脏受累导致,因此尽管异常LGL克隆持续存在,但有效治疗血细胞减少症是有益的。