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成熟B细胞急性白血病:9例临床、形态学、免疫学及细胞遗传学研究

Mature B-cell acute leukemia: a clinical, morphological, immunological, and cytogenetic study of nine cases.

作者信息

Hammami A, Chan W C, Michels S D, Nassar V H

机构信息

Emory University School of Medicine, Department of Pathology, Atlanta, Georgia 30322.

出版信息

Hematol Pathol. 1991;5(3):109-18.

PMID:1960134
Abstract

Nine patients who presented with acute lymphoid leukemia of mature B-cell phenotype without FAB-L3 morphology are discussed. All patients were male with a median age of 69 years. All patients had extensive bone marrow involvement at presentation with lymphoid leukemic cells in the peripheral blood. Six patients had extramedullary disease and 3 developed meningeal involvement sometime during the course of their illness. The leukemic cells were negative for terminal deoxynucleotidyl transferase in all 9 cases, and monoclonal surface immunoglobulin was demonstrated in all 8 cases evaluated with a lambda light chain predominance. Clonal chromosomal abnormalities were detected in 4 of 6 cases studied with no specific pattern identified, although abnormalities involving chromosome 8 were present in all 4 cases. Despite aggressive chemotherapy, only 2 patients achieved complete remission which was of short duration (1 month) in 1 patient. Eight patients died of their disease 1 week to 20 months after diagnosis with a median survival of 5.5 months. Mature B-cell acute leukemias that are not of the FAB-L3 type have a very aggressive clinical course and poor prognosis.

摘要

本文讨论了9例表现为成熟B细胞表型急性淋巴细胞白血病且无FAB-L3形态学特征的患者。所有患者均为男性,中位年龄69岁。所有患者初诊时均有广泛的骨髓受累,外周血中可见淋巴白血病细胞。6例患者有髓外病变,3例在病程中出现脑膜受累。所有9例患者的白血病细胞末端脱氧核苷酸转移酶均为阴性,在所有8例进行评估的患者中均检测到单克隆表面免疫球蛋白,以λ轻链为主。在6例进行研究的患者中,4例检测到克隆性染色体异常,未发现特定模式,尽管所有4例均存在涉及8号染色体的异常。尽管进行了积极的化疗,但只有2例患者达到完全缓解,其中1例缓解期较短(1个月)。8例患者在诊断后1周内至20个月死于疾病,中位生存期为5.5个月。非FAB-L3型的成熟B细胞急性白血病临床病程非常凶险,预后较差。

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