{"title":"成熟b细胞急性白血病:九例临床、形态学、免疫学和细胞遗传学研究。","authors":"A Hammami, W C Chan, S D Michels, V H Nassar","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":77160,"journal":{"name":"Hematologic pathology","volume":"5 3","pages":"109-18"},"PeriodicalIF":0.0000,"publicationDate":"1991-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Mature B-cell acute leukemia: a clinical, morphological, immunological, and cytogenetic study of nine cases.\",\"authors\":\"A Hammami, W C Chan, S D Michels, V H Nassar\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":77160,\"journal\":{\"name\":\"Hematologic pathology\",\"volume\":\"5 3\",\"pages\":\"109-18\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1991-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hematologic pathology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hematologic pathology","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Mature B-cell acute leukemia: a clinical, morphological, immunological, and cytogenetic study of nine cases.
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.