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Clinical and laboratory features of Adult T-cell Leukemia/lymphoma (ATL): a study of 37 cases
Affiliations- Department of Clinical Research, Instituto Estadual de Hematologia Arthur Siqueira Cavalcanti (HEMORIO), Rua Frei Caneca 8, 20211-030, Rio de Janeiro
- Department of Haematology, Instituto Estadual de Hematologia Arthur Siqueira Cavalcanti (HEMORIO), Rua Frei Caneca 8, 20211-030, Rio de Janeiro
- Haematology Laboratory, Instituto Estadual de Hematologia Arthur Siqueira Cavalcanti (HEMORIO), Rua Frei Caneca 8, 20211-030, Rio de Janeiro
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Abstract
Adult T-cell leukemia/lymphoma (ATL) related to the human T-cell lymphotropic virus type I (HTLV-I) is a malignant lymphoproliferative disease. ATL is classified in four subtypes: lymphoma, acute, smoldering and chronic. We analyzed, retrospectively, 46 consecutive patients with T-cell disease with ATL diagnosed from 1995 to 2007. ATL diagnose was confirmed in 37 of these patients. There were 26 females and 11 males (70% vs 30%, respectively, p=0.014). The median age was 42 years old. Twenty-five were nonwhite and twelve were white (67.6% vs 32.4%, respectively, p=0.033).Twenty two patients had the acute form, eight had chronic form and seven had lymphomatous form. Two of them had osteolytic lesions. There were two cases with pulmonary infiltrates; one patient had ATL associated to Hansen’s disease. All cases had antibodies to HTLV-I confirmed by Western Blot, polymerase chain reaction (PCR) was performed in 22 cases. Flow cytometry revealed positivity for CD4 and CD25 in most cases. The mean and median overall survival time was 11.3 months and 2 months, respectively. The family´s study showed that 60% of the mothers were HTLV-I seropositive. These data emphasize the importance of a serologic screening for HTLV and immunophenotyping to differentiate ATL from others T-lymphoproliferative disorders.
Abstract
Adult T-cell leukemia/lymphoma (ATL) related to the human T-cell lymphotropic virus type I (HTLV-I) is a malignant lymphoproliferative disease. ATL is classified in four subtypes: lymphoma, acute, smoldering and chronic. We analyzed, retrospectively, 46 consecutive patients with T-cell disease with ATL diagnosed from 1995 to 2007. ATL diagnose was confirmed in 37 of these patients. There were 26 females and 11 males (70% vs 30%, respectively, p=0.014). The median age was 42 years old. Twenty-five were nonwhite and twelve were white (67.6% vs 32.4%, respectively, p=0.033).Twenty two patients had the acute form, eight had chronic form and seven had lymphomatous form. Two of them had osteolytic lesions. There were two cases with pulmonary infiltrates; one patient had ATL associated to Hansen’s disease. All cases had antibodies to HTLV-I confirmed by Western Blot, polymerase chain reaction (PCR) was performed in 22 cases. Flow cytometry revealed positivity for CD4 and CD25 in most cases. The mean and median overall survival time was 11.3 months and 2 months, respectively. The family´s study showed that 60% of the mothers were HTLV-I seropositive. These data emphasize the importance of a serologic screening for HTLV and immunophenotyping to differentiate ATL from others T-lymphoproliferative disorders.
Keywords
HTLV-I; ATL; Clinical characteristics; Brazil
About this articleTitle
Clinical and laboratory features of Adult T-cell Leukemia/lymphoma (ATL): a study of 37 cases
Journal
Issue
Pages
75-84
Published online
2018-08-30
Page views
144
Article views/downloads
151
DOI
10.2478/ahp-2018-0014
Bibliographic record
Acta Haematol Pol 2018;49(2):75-84.
Keywords
HTLV-I
ATL
Clinical characteristics
Brazil
Authors
Cristiane da de Araújo
Mirna Maira Carmo
Claudia de Maximo
Sílvia Maia de Carvalho