Gill P S, Meyer P R, Pavlova Z, Levine A M
J Clin Oncol. 1986 May;4(5):737-43. doi: 10.1200/JCO.1986.4.5.737.
Acute lymphocytic leukemia (ALL) is a heterogeneous group of disorders, clinically, immunologically, and pathologically. ALL of a B cell phenotype (B-ALL) is the least common. We have studied ten adult patients with B-ALL, none of whom had a tumor mass. The median age was 56 years (range, 30 to 90). A history of an altered immune state was noted in four cases: a distant history of Hashimoto's thyroiditis in one, pregnancy in one, and acquired immunodeficiency syndrome in two. Two patients presented with CNS involvement, and in two additional patients CNS leukemia developed during the course of disease. By the French-American-British (FAB) classification system, L3 leukemic morphology was present in nine, whereas L2 was present in one. Circulating leukemic blasts varied from less than 500/dL to greater than 15,000/dL. Eight patients were thrombocytopenic, and eight were anemic at presentation. Immunologic marker studies on leukemic blasts revealed monoclonal kappa light chain marking in nine and monoclonal lambda in one. Following chemotherapy, complete remission was achieved in three patients, two of whom experienced relapse within 9 months. The median survival for the group was 3 months, and only one patient experienced long-term, disease-free survival. We conclude that B-ALL in the adult presents with the classic L3 morphologic picture in the majority and is associated with extremely short survival.
急性淋巴细胞白血病(ALL)在临床、免疫和病理方面是一组异质性疾病。B细胞表型的ALL(B-ALL)最为少见。我们研究了10例成年B-ALL患者,均无肿瘤肿块。中位年龄为56岁(范围30至90岁)。4例有免疫状态改变史:1例有桥本甲状腺炎病史,1例妊娠,2例获得性免疫缺陷综合征。2例患者出现中枢神经系统受累,另有2例患者在病程中发生中枢神经系统白血病。按照法国-美国-英国(FAB)分类系统,9例为L3白血病形态,1例为L2。循环白血病原始细胞数量从少于500/μL到大于15,000/μL不等。8例患者血小板减少,8例患者就诊时贫血。白血病原始细胞的免疫标志物研究显示,9例为单克隆κ轻链标记,1例为单克隆λ轻链标记。化疗后,3例患者达到完全缓解,其中2例在9个月内复发。该组患者的中位生存期为3个月,只有1例患者获得长期无病生存。我们得出结论,成人B-ALL多数呈现经典的L3形态学表现,且生存期极短。