scholarly journals Clinical and laboratory features of Adult T-cell Leukemia/lymphoma (ATL): a study of 37 cases

2018 ◽  
Vol 49 (2) ◽  
pp. 75-84
Author(s):  
Cristiane da Silva Rodrigues de Araújo ◽  
Mirna Maira Bezerra Calazan do Carmo ◽  
Claudia de Alvarenga Maximo ◽  
Sílvia Maia Farias de Carvalho

AbstractAdult 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.

2006 ◽  
Vol 8 (3) ◽  
pp. 169-176 ◽  
Author(s):  
Abraham M. Y. Nomura ◽  
Eugene T. Yanagihara ◽  
William A. Blattner ◽  
Gloria Y. F. Ho ◽  
Melvin S. Inamasu ◽  
...  

2008 ◽  
Vol 41 (3) ◽  
pp. 288-292 ◽  
Author(s):  
Ricardo Aparecido Olivo ◽  
Fabrício Frederico Mendes Martins ◽  
Sheila Soares ◽  
Helio Moraes-Souza

Adult T-cell leukemia/lymphoma is a lymphoproliferative disorder of mature T lymphocytes associated with infection with human T-cell lymphotrophic virus type I (HTLV-I). Adult T-cell leukemia/lymphoma is characterized by clinical and laboratory polymorphism that allows it to be classified into four distinct subgroups: smoldering, chronic, acute and lymphomatous types. We present here two cases of adult T-cell leukemia/lymphoma, respectively in the acute and lymphomatous forms of the disease. Case 1 was a 35-year-old woman who presented abdominal distension accompanied by hepatosplenomegaly, adenomegaly, skin lesions, positivity for anti-HTLV-I antibodies and leukocytosis with the presence of flower cells. Case 2 was a 38-year-old man who was admitted with generalized lymphadenomegaly, positivity for anti-HTLV-I antibodies, hypercalcemia and osteolytic lesions. In this paper, we correlate the clinical-laboratory findings of these two cases with data in the literature.


1986 ◽  
Vol 83 (12) ◽  
pp. 4524-4528 ◽  
Author(s):  
M. Shimoyama ◽  
Y. Kagami ◽  
K. Shimotohno ◽  
M. Miwa ◽  
K. Minato ◽  
...  

Blood ◽  
1990 ◽  
Vol 76 (5) ◽  
pp. 971-976 ◽  
Author(s):  
SJ Greenberg ◽  
ES Jaffe ◽  
GD Ehrlich ◽  
NJ Korman ◽  
BJ Poiesz ◽  
...  

Abstract Kaposi's sarcoma (KS) developed in a patient with human T-cell leukemia virus type I (HTLV-I)-associated adult T-cell leukemia who was treated with a short-term course of monoclonal antibody immunotherapy. The presentation was transient and temporally related to the underlying clinical course. The association of KS in an HTLV-I infected, but not human immunodeficiency virus (HIV)-infected, individual should alert investigators to the occurrence of KS in retroviral-associated diseases other than acquired immunodeficiency disease syndrome. Recognition of the similarities and differences between HTLV-I and HIV infections may provide insights concerning the angiopathogenesis of KS.


2014 ◽  
Vol 211 (13) ◽  
pp. 2497-2505 ◽  
Author(s):  
Masao Nakagawa ◽  
Roland Schmitz ◽  
Wenming Xiao ◽  
Carolyn K. Goldman ◽  
Weihong Xu ◽  
...  

Adult T cell leukemia/lymphoma (ATLL) is an aggressive malignancy caused by human T cell lymphotropic virus type-I (HTLV-I) without curative treatment at present. To illuminate the pathogenesis of ATLL we performed whole transcriptome sequencing of purified ATLL patient samples and discovered recurrent somatic mutations in CCR4, encoding CC chemokine receptor 4. CCR4 mutations were detected in 14/53 ATLL samples (26%) and consisted exclusively of nonsense or frameshift mutations that truncated the coding region at C329, Q330, or Y331 in the carboxy terminus. Functionally, the CCR4-Q330 nonsense isoform was gain-of-function because it increased cell migration toward the CCR4 ligands CCL17 and CCL22, in part by impairing receptor internalization. This mutant enhanced PI(3) kinase/AKT activation after receptor engagement by CCL22 in ATLL cells and conferred a growth advantage in long-term in vitro cultures. These findings implicate somatic gain-of-function CCR4 mutations in the pathogenesis of ATLL and suggest that inhibition of CCR4 signaling might have therapeutic potential in this refractory malignancy.


2010 ◽  
Vol 207 (13) ◽  
pp. 2785-2792 ◽  
Author(s):  
Hiba El Hajj ◽  
Marwan El-Sabban ◽  
Hideki Hasegawa ◽  
Ghazi Zaatari ◽  
Julien Ablain ◽  
...  

Chronic HTLV-I (human T cell lymphotropic virus type I) infection may cause adult T cell leukemia/lymphoma (ATL), a disease with dismal long-term prognosis. The HTLV-I transactivator, Tax, initiates ATL in transgenic mice. In this study, we demonstrate that an As2O3 and IFN-α combination, known to trigger Tax proteolysis, cures Tax-driven ATL in mice. Unexpectedly, this combination therapy abrogated initial leukemia engraftment into secondary recipients, whereas the primary tumor bulk still grew in the primary hosts, only to ultimately abate later on. This loss of initial transplantability required proteasome function. A similar regimen recently yielded unprecedented disease control in human ATL. Our demonstration that this drug combination targeting Tax stability abrogates tumor cell immortality but not short-term growth may foretell a favorable long-term efficiency of this regimen in patients.


Sign in / Sign up

Export Citation Format

Share Document