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Objective: In Brazil, the National Institute of Cancer estimates for the years 2023-2025 about 12,040 new cases of NHL, about 1,444 of peripheral T-cell lymphomas (PTCLs). T-cell Brazil project is an ambispective study inserting new diagnosis from January 2015 to December 2022. Our goal was to explore a prospective cohort (PC), April 2017-December 2022, analyzing primary refractory and relapse (R/R) PTCLs pts to explore bad factors for overall survival (OS). Methodology: PC enrolled 461 pts who received 1ª treatment line. Descriptive analyses, Kaplan-Meier method, Log-Rank test to compare groups and Cox Regression to identify risk factor for OS using IBM-SPSS software v.24. Results: It was identified 171 (37%) pts, 71% refractory and 29% relapsed. Median mo. from treatment to R/R was 6 mo. (1-49). Overall, 42% received 2nd line treatment and these 11% had to bone marrow transplantation. After a median 17 months (0-51) of follow up, 64% pts had died, and 74% due to lymphoma, 17% infections, 9% toxicities.Refractory pts (HR=2.51, P1 extranodal site (HR=1.76, P=0.01) were associated with a higher risk of death in a Cox Regression. Conclusion: This study confirms outcomes for patients treated according to standards treatment. No difference was found in OS with respect to histology. Results confirm that peripheral T-cell lymphomas patients had dismal outcome after relapse or progression, besides of higher IPI and more than one extranodal site at diagnosis. However, HCT as salvage can possibly prolong life as some studies already indicated.

Objective: T-cell Brazil project started in April 2017 an ambispective study focusing to collecting epidemiological and clinical data from the most frequent subtypes of PTCL, among them the ATL. As of July 2022 T-cell Brazil database contained 81 (16%) ATL out of 520 registered cases. Our goals are to describe demographic and clinical features, analyze the overall and progression-free survival (OS and PFS), and try to identify factors that could influence outcome. Methodology: Brazilian Registry using REDcap Platform by Vanderbilt realized descriptive and bivariate analyses, then it was applied Kaplan-Meier method and log-rank test to obtain survival estimates, and besides that, it was used the Cox Regression to identify any factor that could influence the OS and PFS. Results: The median age was 52 years (24-91); 32 (39%) male; the majority of clinical subtypes were 52% lymphoma type; 81% received chemotherapy. The best response assessment after first-line treatment was: progression or no response in 31%; 26% complete response; 21% partial response, 21% not available (NA) due to death or on treatment; 34% of patients were alive and the 24-month OS and PFS was 33% and 21%, respectively. As predictors for PFS and OS were B symptom and elevated LDH values. Conclusion: This study, even recognizing a limited sample size, highlights the poor prognosis associated with ATL, mainly acute and lymphoma type, with high mortality rates. Hence, apparently, a good shot, it would be one of the bases for the prevention of ATL to establish a disease entity of “chronic active HTLV-1 infection” that defines high-risk carriers for ATL development, and then, enables preventive intervention.

The novel Coronavirus (CoVid-19) outbreak is now consider a world pandemic, affecting more than 1,300,000 people worldwide. Cancer patients are in risk for severe disease, including a higher risk of intensive care unit (ICU) admission, need for invasive ventilation or death. Management of patients with lymphoid malignancies can be challenging during the outbreak, due to need of multiple hospital visits and admissions, immunosuppression and need for chemotherapy, radiotherapy and stem cell transplantation. In this article, we will focus on the practical management of patients with lymphoid malignancies during the COVID-19 pandemic, focusing on minimizing the risk for patients.

Identificação precoce e o tempo para iniciar terapia na sepsis e na neutropenia febril (NF) são fundamentais para redução de mortalidade. Protocolos clínicos gerenciados (PC) são altamente recomendados em ambas as condições. Embora haja medidas comuns nestes PCs (estabilização clínica, coleta de culturas, início de terapia na primeira hora), há poucos dados poucos de lactato sérico na NF. Nossa instituição tem dois PC independentes: PC sepsis e PC NF. Todo paciente que desenvolve NF é manejado dentro do PC NF. O PC Sepsis prevê o acompanhamento de todo paciente com critério de alerta ou com antimicrobiano de amplo espectro. Por este último critério, paciente com NF mesmo sem sinais de alerta estão sujeitos ao PC Sepsis, incluindo coleta de lactato sérico e a mensuração de escores de gravidade. Neste estudo comparamos pacientes submetidos a TMO autólogo manejados dentro do PC Sepsis com aqueles manejados apenas dentro do PC NF. Métodos: Estudo de coorte em pacientes submetidos a TMO autólogo entre 2016 e 2021. Consideramos os seguintes desfechos: infecção de corrente sanguínea (ICS), transferência para terapia intensiva (CTI), tempo de internação, tempo de uso de antimicrobianos, troca da terapia para meropenem e mortalidade. Resultados: Foram revistos 461 pacientes submetidos a Auto TMO (mediana de idade de 56 anos), destes 423 (92%) desenvolveram NF e foram analisados. PC sepsis foi aplicado em 321 (76% dos eventos NF), e os demais 102 (22% das NF) foram manejados apenas pelo PC NF. Não houve diferença em idade, doença de base ou estratégia empírica inicial da NF entre PC sepsis e PC NF. Nos pacientes manejados no PC sepsis, a mediana do LactNF foi 12,8 mg/dL (variando de 1,7 - 57). LactNF >18 e > 20 mg/dL ocorreu em 77 (24%) e 60 (19%) dos casos. LactNF >18 e >20 foram marcadores de transferência para CTI (p < 0.05 para ambos), mas não se associaram a ICS, tempo de internação, tempo de uso de antimicrobiano ou mortalidade. Paciente no PC sepsis tiveram maior uso de meropenem (50% x 33%, p = 0,004) e tempo de uso de antimicrobiano total (7,8 vs. 6,9 dias; p = 0,06). ICS (21% x 26%, p = 0,40) e o tempo de internação após o TMO foram equivalentes (13 vs. 12 dias, p = 0,14) entre PC Sepsis e PC NF. Quatro pacientes falecerem (0,9%), todos no PC sepsis. Embora a aplicação do lactato sérico no momento da neutropenia febril tenha demostrado associação com CTI, não houve melhoria nos desfechos da NF com a aplicação do PC Sepsis quando comparado ao PC NF.

Objective: T-cell Brazil Project was designed as an ambispective data collection from January 2015 to December 2022 of previously untreated patients diagnosed with Peripheral T-cell lymphoma (PTCL) or NK/T-cell lymphoma according to the revised WHO 2017 classification in Brazil. The primary and secondary end points were 2-year overall survival (OS) and progression-free survival (PFS). Clinical, treatment and survival data were also correlated. Methodology: Twenty centers got approved for the study from the local and national institutional review board and registered their cases only online. OS was calculated from diagnosis date until last seen or death date, whereas PFS until first event, progression / relapse, date of death or last seen. Kaplan-Meier method was applied and a Log-rank test to compare their curves. P-value less than 5% was considered. From a total of 416 patients with PTCL, 46 (11%) were diagnosed as AITL. Results: The median age was 65 years (31-82), with 63% males, 94% had advanced-stage disease. All patients received 61% CHOEP, 28% CHOP and 11% CT without anthracycline. 20% of pts were consolidated with autologous transplant (HSCT). There were 19 (41%) deaths, 10 by lymphoma, 8 infections, 1 new neoplasia. With 8-mo median f/u (1-36), OS at 24-mo was 27% and 2-year PFS was 21%. As consolidation, OS was 71% HSCT group vs. 16% no HSCT (P= 0.06) and PFS was 71% vs. 8%, respectively (P= 0.01). Conclusion: These analyses are preliminaries but show a poor outcome of AITL in our population. Most patients were treated with anthracycline-containing combination chemotherapy and just 20% received autologous HSCT. A dismal survival was shown for those who did not receive HSCT.

O cromossomo Filadélfia (Ph1) é a alteração citogenética mais comum da Leucemia Linfoblástica Aguda do adulto (LLA). Esta alteração citogenética predomina nos adultos com mais de 50 anos e na LLA de origem na célula B, principalmente CD10 positiva. O diagnóstico requer a análise citogenética e a pesquisa do mRNA do gene BCR-ABL no sangue periférico ou na medula óssea. A LLA Ph1 apresenta uma sobrevida global em cinco anos inferior a 20% quando tratada com protocolos para LLA. Os poucos casos de cura ocorrem nos pacientes submetidos ao transplante alogênico de medula óssea (TMO). A adição do imatinibe à quimioterapia resultou em melhora na taxa de remissão completa, maior taxa de remissão molecular completa, maior número de pacientes aptos para realizar o TMO, uma maior sobrevida livre de eventos e maior sobrevida global, embora o tempo de seguimento seja ainda muito curto. Entretanto, a taxa de recaídas e o aparecimento de mutações do BCR-ABL resistentes ao imatinibe ainda são preocupantes. No futuro, novos inibidores de tirosina quinase poderão ser incorporados ao tratamento da LLA Ph1.The Philadelphia chromosome (Ph1) is the most frequent abnormality in acute adult lymphoblastic leukemia (ALL). Ph1 positive ALL is more frequent in over 50-year-old adults, in B-cell ALL and CD10-positive ALL. Diagnosis is based on the identification of the BCR-ABL gene mRNA in peripheral blood or bone marrow. The 5-year overall survival of patients with Ph1 positive ALL treated with chemotherapy alone is less than 20%. A few cases may be cured by allogeneic stem cell transplantation. The addition of imatinib to the chemotherapeutic treatment has resulted in more complete remissions, more complete molecular responses, more patients able to perform stem cell transplantation, better event-free survival and better overall survival, although the study follow-up period is very short so far. High relapse rates and the emergence of BCR-ABL mutants resistant to imatinib are still significant. In the future, newer tyrosine-kinase inhibitors may be added to the chemotherapy.

ABSTRACT Chronic lymphocytic leukemia is characterized by clonal proliferation and progressive accumulation of B-cell lymphocytes that typically express CD19+, CD5+ and CD23+. The lymphocytes usually infiltrate the bone marrow, peripheral blood, lymph nodes, and spleen. The diagnosis is established by immunophenotyping circulating B-lymphocytes, and prognosis is defined by two staging systems (Rai and Binet) established by physical examination and blood counts, as well as by several biological and genetic markers. In this update, we present the recommendations from the Brazilian Group of Chronic Lymphocytic Leukemia for the diagnosis and treatment of chronic lymphocytic leukemia. The following recommendations are based on an extensive literature review with the aim of contributing to more uniform patient care in Brazil and possibly in other countries with a similar social-economic profile.