HTLV-I infection among relatives of patients with adult T-cell leukemia/lymphoma in Brazil: analysis of infection transmission.

We examined the presence of HTLV-I infection among 66 family members of 13 patients with well documented ATL to investigate the routes of HLTV-I transmission in a Southeast region of Brazil. HTLV-I infection was screened by an enzyme immunossay (ELISA) test and all repeatedly positive or indeterminate ELISA samples were further tested by a Western-Blot (WB) technique. Indeterminate and inconclusive WB samples were confirmed by a polymerase chain reaction (PCR). ELISA results showed that 40 (60.6%) individuals were not infected; 16 (24.2%) were positive; and 10 (15.2%) were undetermined. Among 16 ELISA positive subjects, 14 (87.5%) were confirmed to be positive by WB while 2 (12.5%) showed inconclusive results. Based on the laboratory data, questionnaire analysis, and family/epidemiological studies, we concluded that HTLV-I vertical transmission occurred in 6 of the 13 families. In 3 of these 6 families, the horizontal transmission also could be demonstrated. An isolated horizontal transmission was detected in one family, and in 6 families we did not find any infected family member. All HTLV-I-infected persons were clinically asymptomatic. The occurrence of an effective HTLV-I vertical transmission detected by the present study suggest that HTLV-I infection is endemic in the Southeast region of Brazil. Consistent with the modes of transmission, the HTLV-I antibody seroprevalence was greater in relatives of ATL patients than in the general blood donor Brazilian population (0.4%). In addition, the present data suggest that HTLV-I carries a high infectivity rate but a low virulence.

Adult T-cell leukemia/lymphoma and cluster of HTLV-I associated diseases in Brazilian settings.

We studied the transmission routes of human T-cell lymphotropic virus type I (HTLV-I) within families of 82 Brazilian patients diagnosed with adult T-cell leukaemia/lymphoma (ATL). Diagnosis of ATL in 43 male and 39 female patients was based on clinical and laboratory criteria of T-cell malignancy and detection of HTLV-I monoclonal integration. Samples were tested for HTLV antibodies and infection was confirmed as HTLV-I by Western Blot and/or polymerase chain reaction (PCR) assays. Overall 26/37 (70%) of mothers, 24/37 (65%) of wives, 8/22 (36%) of husbands, 34/112 (30%) of siblings and 10/82 (12%) offspring were HTLV-I infected. In 11 ATL patients, mothers were repeatedly HTLV-I seronegative, but HTLV-I pol or tax sequences were detected in 2 out of 6 cases tested by PCR. ATL patients with seronegative mothers related the following risk factors for HTLV-I infection: 6 were breast-fed by surrogate mothers with unknown HTLV-I status, 4 had a sexually promiscuous behaviour and 1 had multiple blood transfusions at a young age. Familial aggregation of ATL and other HTLV-I associated diseases such as HTLV-I myelopathy (HAM/TSP) and or uveitis, ATL in sibling pairs or in multiple generations was seen in 9 families. There were 6 families with ATL and TSP sibling pairs. In 3 families at least one parent had died with lymphoma or presenting neurological diseases and 2 offspring with ATL. Further to the extent of vertical and horizontal transmission of HTLV-I infection within ATL families, our results demonstrate that mothers who provide surrogate breast-milk appear to be an important source of HTLV-I transmission and ATL development in Brazil.

Post-transplant lymphoproliferative disorders (PTLD) after renal transplantation: management and evolution of seven cases among 1002 renal transplants in São Paulo, Brazil.

We reported seven cases (0.7%) of PTLD among 1002 renal transplants performed at Renal Transplant Service from Hospital São Paulo-Universidade Federal de São Paulo/Escola Paulista de Medicina, São Paulo, Brazil, between 1976 and 1997. There were three male and four female patients with median age of 37 year-old. According to Ann Arbor staging system there were four localized extra-nodal intermediate-grade NHL, one disseminated low-grade NHL and two polyclonal lymphoid hyperplasia. Four patients received cadaveric, two received related and one received unrelated renal transplant. PTLD occurred after a median latency period of 36 months (ranging from 5 to 84 months). In situ hybridization for EBER1 was performed in five patients and molecular evidence of EBV was found in 3 cases (two DLCL and one lymphoplasmocytoid lymphoma). All patients were treated with immunosuppression withdrawal, four patients received anthracyclin-based chemotherapy for control of localized or systemic clonal disease and three were treated with resection of primary PTLD. Four of seven patients (57%) are in complete remission 11, 20, 25 and 79 months after PTLD onset. One patient lost follow-up and two patients died due to lymphoma relapse, respectively 4 and 10 months after completion of treatment. In conclusion, our experience with this small group of patients showed that: 1) immunosuppression withdrawal is not necessarily associated with loss of renal transplant and can be used as the only treatment for polyclonal PTLD; 2) chemotherapy can simultaneously lead to clonal PTLD remission and periodic immunosuppression, avoiding graft rejection after immunosuppression withdrawal.

Clinical management of six cases of low-risk primary tonsillar non-Hodgkin's lymphoma.

CONTEXT: There have been many reports that favor aggressive systemic treatment with chemotherapy and radiotherapy, even for well-localized lymphomas, avoiding the need for tonsillectomy of the normal tonsil. CASE REPORT: We report six cases of primary tonsillar lymphoma with a median patient age of 42 years. There were two lymphoma cases with diffuse large cells, two cases with mixed small and large cells, one with small cells and one indeterminate. They were treated with six cycles of chemotherapy and cervical radiotherapy. All patients achieved durable complete remission. Our data agree with previous reports that suggested that primary tonsillar high-grade B-cell NHL has a good prognosis if aggressively treated.

Geographic diversity of adult t-cell leukemia/lymphoma in Brazil. The Brazilian ATLL Study Group.

We describe 195 cases of adult T-cell leukemia/lymphoma (ATLL) reported to the national registry of T-cell malignancies in Brazil between 1994 and 1998. We compared the effect of demographic differences and clinical features of 150 consecutive ATLL cases in different regions of this diverse country. At diagnosis, the predominant clinical sub-type was the acute type (60%), followed by lymphoma (22%), chronic (10%) and smoldering (8%) types. Although we expected that different sub-types would be present in different regions, on the basis of immunogenetic factors determined by ethnicity, we did not demonstrate these differences. There were no significant differences among ATLL subtypes by age or gender. No ethnic group predominated in the total population of patients, but significant differences were noted when examining ethnic distribution by region. Reflecting the general population distribution, white patients were seen more often in São Paulo and black patients in Bahia, than in other regions. In most regions, cases were equally distributed between blacks and mulattos, except in Pernambuco, where blacks were less frequent. The main clinical features were lymphadenopathy, skin lesions, hypercalcemia and hepatomegaly. Fourteen patients (9%) suffered from HTLV-I-associated myelopathy (HAM/TSP), either at diagnosis or during follow-up of ATLL. All cases but one had antibodies to HTLV-I, with concordant results with ELISA, WB and PCR analyses. For the antibody-negative case, pol and tax gene sequences were present in tumor cells when subjected to PCR analyses. The prognosis was generally poor, suggesting that the disease in Brazil behaves in similar fashion regardless of ethnic or geographical differences.

Pacientes adultos com leucemia aguda - experiência da Faculdade de Medicina do ABC / Adult patients with acute leukemia - ABC Medical School experience

Introdução: Houve uma melhora lenta, porém definitiva no tratamento das leucemias agudas nos últimos anos. No entanto, existem controvérsias para pacientes com características específicas. Este trabalho tem como objetivo descrever o perfil epidemiológico dos pacientes com leucemia aguda atendidos no Hospital Estadual Mário Covas (HEMC), assim como demonstrar as curvas de sobrevida global e livre de doença e as taxas de mortalidade desta população. Métodos: Realizado estudo de corte transversal para avaliação de pacientes com diagnóstico de leucemias agudas da FMABC atendidos, no HEMC, no período de 2008 a 2012. Análise estatística das curvas de sobrevida utilizando Kaplan-Meier (XL Stat® v2012). Resultados: LMA: 50 pacientes com média de 57,72 anos de idade (18 a 89 anos), predomínio do sexo feminino 2,12: 1 e SLD = SG = 42 meses (média de seguimento = 49,9 meses); sendo 12 pacientes com LMA-M3 (SG=SLD=50%) e 38 outros subtipos (SG=SLD=39,5%). LLA: 17 pacientes, com média de 33,7 anos de idade (19 a 66 anos), predomínio do sexo masculino 1,43: 1 e SG = 58,82%, SLD = 47,3% (média de seguimento = 24,6 meses). Conclusão: Pacientes com LMA que atingem resposta na fase de indução apresentam curvas de SG e SLD compatíveis com a literatura. Pacientes com LLA apresentam SG e SLD inferior ao observado na literatura. É preciso prosseguir com este programa a fim de determinar o melhor tratamento para estes pacientes e o impacto do TCTH, assim como sua viabilidade.

Granuloma letal da linha média: abordagem clínica e terapêutica de três casos / Lethal midline granuloma: clinical management of three cases

Objetivo. Relato de três casos do GLLM acompanhados pela Disciplina de Hematologia e Hemoterapia da Unifesp-EPM que tiveram boa resposta à terapêutica e evoluçao favorável. Métodos. Após confirmaçao histológica e histoquímica, os pacientes foram submetidos à tratamento quimio e radioterápico com boa resposta terapêutica. Resultados. Atualmente estes pacientes encontram-se em remissao total da doença, com sobrevida média de 45 meses. Conclusao. Levando-se em consideraçao nossa pequena experiência, acreditamos que o tratamento radioterápico e a abordagem quimioterápica inicial agressiva sao fundamentais para uma boa evoluçao deste tipo de linfoma.