Assuntos
Angiografia por Tomografia Computadorizada/métodos , Ciclofosfamida/administração & dosagem , Leucemia Prolinfocítica de Células T , Prednisona/administração & dosagem , Fibrose Retroperitoneal , Vasculite Sistêmica , Vidarabina/análogos & derivados , Idoso , Antineoplásicos/administração & dosagem , Aorta Torácica/diagnóstico por imagem , Dor no Peito/diagnóstico , Dor no Peito/etiologia , Dor no Peito/terapia , Vasos Coronários/diagnóstico por imagem , Humanos , Leucemia Prolinfocítica de Células T/fisiopatologia , Leucemia Prolinfocítica de Células T/terapia , Linfadenopatia/diagnóstico , Linfadenopatia/etiologia , Linfadenopatia/terapia , Masculino , Fibrose Retroperitoneal/diagnóstico , Fibrose Retroperitoneal/etiologia , Fibrose Retroperitoneal/fisiopatologia , Fibrose Retroperitoneal/terapia , Vasculite Sistêmica/diagnóstico , Vasculite Sistêmica/etiologia , Vasculite Sistêmica/fisiopatologia , Vasculite Sistêmica/terapia , Resultado do Tratamento , Vidarabina/administração & dosagemRESUMO
T-cell prolymphocytic leukemia (T-PLL) is a rare type of peripheral T-cell leukemia. In this study, we examined the clinical and biological characteristics of 11 Japanese patients with T-PLL. Median age was 74 years, with male predominance. Median lymphocyte frequency was 85.3% in blood. Physical characteristics were splenomegaly (36.4%), tiny lymph adenopathy (63.6%), skin lesion (9.1%) and pleural effusion (27.3%). Median survival was 30.1 months, despite treatment with various chemotherapeutic modalities. Although complex chromosomal abnormalities were observed in 5 of 11 cases (45.5%), typical 14q32 and Xq28 abnormalities were not detected. TCL1A mRNA expression was observed in 6 of 11 cases (54.5%) on real-time quantitative PCR. In 5 of these 6 cases, flow cytometric analysis and/or immunohistochemistry confirmed the expression of TCLA1 protein. Split signals for the TCL1 region on fluorescence in situ hybridization confirmed rearrangement in 3 out of 7 cases evaluated. These cases corresponded to cases that were positive for TCL1A expression, suggesting that rearrangement of the TCL1 region induced high expression of TCL1A gene. In summary, a substantial number of T-PLL cases in Japan had abnormal expression of TCL1A, probably due to rearrangement of TCL1 region. Expression and/or rearrangement of TCL1A may, therefore, be a useful marker for diagnosing T-PLL, regardless of chromosomal abnormalities.
Assuntos
Regulação Leucêmica da Expressão Gênica , Rearranjo Gênico , Leucemia Prolinfocítica de Células T/genética , Leucemia Prolinfocítica de Células T/metabolismo , Proteínas Proto-Oncogênicas/genética , Proteínas Proto-Oncogênicas/metabolismo , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Estudos de Associação Genética , Humanos , Japão , Leucemia Prolinfocítica de Células T/mortalidade , Leucemia Prolinfocítica de Células T/fisiopatologia , Masculino , Pessoa de Meia-Idade , Análise de SobrevidaRESUMO
T-cell prolymphocytic leukemia (T-PLL) is typically associated with an aggressive clinical course, with a median survival of less than 1 year. We report a case of T-PLL that displays multiple cytogenetic abnormalities, with the most complex subclone having the following karyotype: 47, Y, -X, +8, inv (10) (p12q26), del (11) (p13p15) +marker. However, despite this genetic complexity, the leukemia has behaved in a remarkably indolent manner, with the patient remaining asymptomatic, without therapeutic intervention, for more than 7 years. The unusually benign behavior of this disease calls into question the validity of grouping such cases under the umbrella of T-PLL and warrants a reconsideration of T-cell chronic lymphocytic leukemia (no longer recognized as a distinct disease) as a bona fide diagnostic entity.
Assuntos
Leucemia Prolinfocítica de Células T/genética , Leucemia Prolinfocítica/genética , Idoso , Análise Citogenética , Diagnóstico Diferencial , Humanos , Cariotipagem , Leucemia Prolinfocítica/diagnóstico , Leucemia Prolinfocítica/fisiopatologia , Leucemia Prolinfocítica de Células T/diagnóstico , Leucemia Prolinfocítica de Células T/fisiopatologia , MasculinoRESUMO
We report a case of T-cell chronic lymphocytic leukemia (T-CLL) in which the lymphocytes were small and mature, did not have cytoplasmic granulation, and appeared to be similar to those of B-cell chronic lymphocytic leukemia (B-CLL). Immunophenotyping of the lymphocytes revealed CD2+, CD4+, CD5+, and CD25+ on the cell surface, but expression of the CD3 and TCR-alpha beta molecules was localized only in the cytoplasm. The clinical course was as indolent as that of B-CLL, and the patient was able to undergo open-heart surgery. Thus, we confirmed that true T-CLL is able to exist as a counterpart of B-CLL.
Assuntos
Complexo CD3/imunologia , Leucemia Prolinfocítica de Células T/imunologia , Receptores de Antígenos de Linfócitos T alfa-beta/imunologia , Idoso , Antígenos de Superfície/imunologia , Complexo CD3/biossíntese , Citoplasma/imunologia , Diagnóstico Diferencial , Humanos , Imunofenotipagem , Leucemia Linfocítica Crônica de Células B/imunologia , Leucemia Linfocítica Crônica de Células B/fisiopatologia , Leucemia Prolinfocítica de Células T/fisiopatologia , Masculino , Receptores de Antígenos de Linfócitos T alfa-beta/biossínteseRESUMO
A 70-year-old woman presented with bilateral anterior uveitis. She was on a gluten-free diet because of a celiac disease which had been diagnosed 3 months before. An anterior chamber aspirate contained a majority of large granular lymphocytes (LGL). The investigation of a chronic neutropenia led to the diagnosis of an otherwise typical T-LGL leukemia. This seems to be the first report of a CD3+ CD4- CD8+ T-LGL leukemia causing anterior uveitis through infiltration of leukemic cells, and the second report of an intriguing association of celiac disease with T-LGL leukemia.
Assuntos
Doença Celíaca/complicações , Leucemia Prolinfocítica de Células T/complicações , Uveíte Anterior/etiologia , Idoso , Feminino , Humanos , Leucemia Prolinfocítica de Células T/fisiopatologia , Neutropenia/etiologia , Neutropenia/fisiopatologiaRESUMO
The capacity to generate lymphokine-activated killer (LAK) cells and the susceptibility of the neoplastic cells to both allogeneic and autologous LAK effectors were studied in B and T chronic lymphoproliferative disorders. While in B-cell chronic lymphocytic leukemia (B-CLL) the depressed natural killer function could be restored after a 7-day incubation with recombinant interleukin (IL-2), B-CLL mononuclear cells showed a reduced LAK activity compared with normal LAK cells. Furthermore, in all but 1 of the 20 B-CLL samples tested the leukemic cells were totally resistant to autologous LAK effectors. In most cases the leukemic cells were also resistant to normal allogeneic LAK cells. Competition experiments demonstrated that the patients' LAK cells, as well as normal LAK effectors, were capable of recognizing B-CLL cells, pointing, therefore, to a postbinding cytolytic defect. In hairy cell leukemia (HCL) an overall reduced LAK activity against allogeneic targets was documented, but, at variance from B-CLL, hairy cells were often susceptible to the lytic effect of normal LAK cells, and in half of the cases tested the neoplastic population was also sensitive in an autologous system. Similarly to B-CLL, in the great majority of T chronic lymphoproliferative disorders studied, the pathologic cells were resistant to normal and autologous LAK effectors and a defective LAK generation was found. These results demonstrate that in most B and T chronic leukemias the LAK function is defective and, when inducible, does not appear directed against the leukemic population. The possibility of exploiting an immunotherapeutic approach with IL-2/LAK cells in the management of chronic lymphoproliferative disorders does not gain support by these findings.