How to manage lymphoid malignancies during novel 2019 coronavirus (CoVid-19) outbreak: a Brazilian task force recommendation

ABSTRACT: 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.

Linfoma anaplásico de células grandes associado a implante mamários: um desafio diagnóstico / Breast implant-associated anaplastic large cell lymphoma: a diagnostic challenge

O linfoma anaplásico de células grandes associado a implante mamário (BIA-ALCL ) é uma entidade provisória com características morfológicas e imunofenotípicas indistinguíveis do linfoma anaplásico de células grandes (ALCL) ALK negativo. Ao contrário do ALCL, o BIA-ALCL surge principalmente em associação ao implante mamário. A confirmação diagnóstica do BIA-ALCL pode ser difícil e a associação de características morfológicas e patológicas com citometria de fluxo e imuno-histoquímica pode auxiliar no diagnóstico. O objetivo deste relatório é descrever um caso de BIA-ALCL no qual a análise citológica e imunofenotipológica utilizando citometria de fluxo sugeriu a presença de grandes células positivas para CD30 no líquido de derrame.

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a provisional entity with morphological and immunophenotypic characteristics indistinguishable from ALKnegative anaplastic large cell lymphoma (ALCL). Unlike ALCL, BIA-ALCL arises mainly in association with breast implantation. Diagnostic confirmation of BIA-ALCL can be difficult and associating morphological and pathological hallmarks with flow cytometry and immunohistochemistry can assist in the diagnosis. The objective of this report is to describe a case of BIA-ALCL in which cytological and immunophenotypological analysis using flow cytometry suggested the presence of large CD30-positive cells in the effusion fluid.

Evaluación longitudinal de la mucositis oral en el trasplante de células madre hematopoyéticas: un estudio piloto / Longitudinal evaluation of oral mucositis in hematopoietic stem cell transplantation: a pilot study

Resumen Objetivo: Describir la incidencia y el puntaje de la mucositis oral (MO) y las morbilidades relacionadas en individuos sometidos a trasplante de células madre hematopoyéticas (TCMH) a lo largo del período de inmunosupresión. Métodos: Los sujetos con enfermedades onco / hematológicas, mayores de 14 años, sometidos a TCMH alogénico fueron evaluados diariamente por la presencia y clasificación de OM, nivel de dolor, disfagia, disgeusia y xerostomía. El examen comenzó dos días antes de la infusión de células madre hematopoyéticas y finalizó veinte días después. La OM se clasificó de acuerdo con la escala de la OMS y se utilizó la escala analógica visual (EVA) para medir el nivel de dolor. Resultados: Se reclutaron 23 individuos, el 83% con enfermedades malignas y el 91% con OM. La mediana del grado máximo de OM fue 3 y el nivel máximo de dolor fue 9. Hubo una mediana de 11 días de uso de medicación opioide. Los sujetos que tuvieron el mayor número de días con dolor en la boca alcanzaron el grado máximo de OM y el mayor número de días y el uso de opioides. Conclusión: Hubo una alta incidencia y puntuaciones más altas de OM, pérdida de masa corporal y dolor en esta muestra.

Abstract Aim: To describe the oral mucositis (OM)` incidence and score, and related morbidities in individuals submitted to Hematopoietic Stem Cell Transplantation (HSCT) throughout the immunosuppression period of time. Methods: Subjects with onco / hematological diseases, older than 14 years, submitted to allogeneic HSCT were daily evaluated by the presence and classification of OM, pain level, dysphagia, dysgeusia and xerostomia. The examination started two days before the infusion of hematopoietic stem cells and ended twenty days later. The OM was classified according to the WHO scale and visual analog scale (VAS) was used to measure pain level. Results: Twenty-three individuals were recruted, 83% with malignant diseases and 91% had OM. The median of maximum OM degree was 3 and the maximum pain level was 9. There was a median of 11 days of opioid medication use. The subjects who had the highest mean number of days with mouth pain reached the maximum degree of OM and higher number of days and opiod use. Conclusion: There was a high incidence and high scores of OM, loss of body mass and pain in this sample.

THPO gene variants in patients with acquired aplastic anemia

ABSTRACT Background: Human aplastic anemia is a hematologic disease characterized by low peripheral blood cell counts associated with reduced numbers of hematopoietic stem and progenitor cells and a hypocellular bone marrow. Thrombopoietin (THPO) regulates megakaryocytes, but it also stimulates hematopoietic stem and progenitor cells. Biallelic mutations in the THPO gene have been reported in a family with recessive inherited aplastic anemia. Methods: This study screened 83 patients diagnosed with acquired aplastic anemia and 92 paired healthy controls for germline variants in the THPO gene using Sanger sequencing. Results: Three common single nucleotide polymorphisms were identified in patients and controls at comparable allele frequencies. There was no correlation between the single nucleotide polymorphism carrier status and platelet counts at diagnosis. Conclusion: The presence of THPO polymorphisms is comparable between patients with acquired aplastic anemia and healthy individuals.

Qualidade de vida do paciente com neoplasia hematológica submetido à quimioterapia / The quality of life of patients with hematological neoplasia undergoing chemotherapy / Calidad de vida del paciente con neoplasma hematológica sometido a quimioterapia

Este estudo teve por objetivo analisar a qualidade de vida do paciente com neoplasia hematológica submetido à quimioterapia. Realizou-se pesquisa quantitativa a partir da aplicação do questionário genérico WHOQOL-bref. A amostra foi composta de 16 pacientes do setor de ambulatório de quimioterapia de alto risco de um hospital de ensino do município de Curitiba-PR, no período de fevereiro a abril de 2010. Os resultados evidenciaram a prevalência do sexo masculino e a média de idade dos participantes esteve entre 20 e 64 anos. Entre os tipos de neoplasias hematológicas encontraram-se 46,7% de leucemia linfocítica aguda, 33,3% de leucemia mieloide aguda e 20% entre linfoma não hodgkin, mieloma múltiplo e tricoleucemia. O período de tratamento foi de duas semanas a 24 meses e o número de sessões de quimioterapia foi entre uma e 80. Mediante análise, pode-se inferir que os domínios físicos e psicológicos foram os que sofreram mais alteração, porém sem diferença estatisticamente significativa para todos os domínios. Ressalta-se que o câncer altera indiscutivelmente todos os aspectos da vida do indivíduo e acarreta profundas alterações na sua rotina e hábitos de vida. Neste sentido, observam-se a necessidade e a importância do cuidado da enfermagem na intervenção desse processo.

This study aimed to analyze the quality of life of patients with hematological neoplasia undergoing chemotherapy. A quantitative research wascarried out based on the application of the WHOQOL-bref generic questionnaire. The sample was composed of 16 patients in the outpatient highrisk chemotherapy sector in a teaching hospital in the city of Curitiba-PR between February and April of 2010. The results showed a prevalence ofmales with ages between 20 and 64 years old. Among the types of hematological neoplasias, 46.7% were acute lymphocytic leukemia, 33.3% wereacute myelogenous leukemia, and 20% were divided between non-Hodgkin’s lymphoma, multiple myeloma, and hairy cell leukemia. The treatmentperiod varied between 2 weeks and 24 months, and the number of chemotherapy sessions between 1 and 80. The analysis showed that thephysical and psychological domains suffered the most changes, however, no statistically significant difference between all domains was observed.Cancer arguably changes every aspect of an individual’s life and brings profound changes in their routine and habits of life. Thus, the necessity andimportance of nursing care intervention in this process is observed.

Este estudio tuvo como objetivo analizar la calidad de vida del paciente con neoplasia hematológica sometido a quimioterapia. Se realizó una investigación cuantitativa a través de la aplicación del cuestionario genérico WHOQOL - bref. La muestra estuvo compuesta de 16 pacientes del sector ambulatorio de quimioterapia d alto riesgo de un Hospital Universitario del municipio de Curitiba/PR, entre Febrero y Abril de 2010. Los resultados mostraron la prevalencia del sexo masculino, edad de los participantes entre 20 y 64 años. Entre los tipos de neoplasias hematológicas se encontró 46,7% de leucemia linfocítica aguda, 33,3% leucemia mieloide aguda y 20% entre linfoma no-hodgkin, mieloma múltiple y tricoleucemia. El periodo de tratamiento varió entre dos semanas y 24 meses y el número de sesiones de quimioterapia entre una y 80. Mediante análisis se puede inferir que los dominios físicos y psicológicos fueron los que sufrieron más alteraciones, pero sin diferencia estadísticamente significativa para todos los dominios. Se realza que la presencia del cáncer altera indiscutiblemente todos los aspectos de la vida del individuo y que lleva a profundas alteraciones en su rutina y costumbres de vida. En este sentido, observamos la necesidad y la importancia del cuidado de enfermería en la intervención de este proceso.

Transplante de células-tronco hematopoéticas em crianças e adolescentes com leucemia aguda: experiência de duas instituições brasileiras / Hematopoietic stem cell transplantation in children and adolescents with acute leukemia: experience of two Brazilian institutions

O transplante de células-tronco hematopoéticas (TCTH) é o tratamento de escolha para leucemias agudas de alto risco. Apesar da melhora na sobrevida destes pacientes, a recidiva continua sendo a maior causa de óbito pós-transplante de células-tronco hematopoéticas. O objetivo deste trabalho foi analisar os resultados dos transplantes realizados em crianças com leucemia aguda em duas instituições brasileiras. Realizou-se estudo retrospectivo de 208 pacientes transplantados entre 1990-2007. Mediana de idade: 9 anos; 119 pacientes com leucemia linfoide aguda (LLA) e 89 com leucemia mieloide aguda (LMA). Doença precoce: CR1 e CR2. ... 14/195 pacientes tiveram falha primária de pega (8 por cento). Não houve diferença na sobrevida global e sobrevida livre de recaída entre pacientes com leucemia linfoide aguda e leucemia mieloide aguda, entre transplantes aparentados e não aparentados, tampouco entre as fontes de células utilizadas. O desenvolvimento da doença do enxerto contra hospedeiro (DECH) aguda ou crônica também não influenciou a sobrevida global e sobrevida livre de recaída. Pacientes com leucemia linfoide aguda condicionados com irradiação corporal total (TBI) apresentaram melhor sobrevida global e sobrevida livre de recaída (p<0,001). Cento e dezoito pacientes morreram entre 1-1.654 dias pós-transplante de células-tronco hematopoéticas (M:160). Mortalidade relacionada a transplante (MRT) (dia+100): 16 por cento. Incidência cumulativa de recaída: 40 por cento (3 anos). Pacientes com doença avançada tiveram menor sobrevida global e sobrevida livre de recaída (três anos)(p<0,001). Na análise multivariada, o status da doença foi o principal fator associado ao aumento da sobrevida global e sobrevida livre de recaída. Nossos resultados mostram que é possível se atingir uma boa sobrevida para pacientes com doença precoce e também mostram a baixa eficácia naqueles com doença avançada.

Hematopoietic Stem Cell transplantation (HSCT) is the treatment of choice for patients with high-risk leukemia. In spite of this, relapse remains a major cause of death of these patients. Our objective was to analyze the outcomes of patients with acute leukemia submitted to hematopoietic stem cell transplantation in two Brazilian institutions... There were no differences in the overall survival and event free survival between patients with acute lymphocytic leukemia and acute myeloid leukemia, between sources of cells used or between those who developed acute or chronic graft-versus-host disease (GVHD). When comparing transplants from related and unrelated donors, there was no difference in the overall survival. Patients with acute lymphocytic leukemia receiving the total body irradiation (TBI) conditioning regimen had better overall survival and event free survival (p<0.001). One hundred and eighteen patients died between 0 and 1654 days after hematopoietic stem cell transplantation (M: 160 days). Transplantation-related-mortality (TRM) at D+100 was 16 percent and cumulative incidence of relapse was 40 percent (3 years). Patients with advanced disease had lower 3-year overall survival and event free survival (p<0.001). Multivariate analysis showed that disease status was the most significant factor associated with higher event free survival and overall survival . Our results show that children and adolescents transplanted with early disease can achieve considerable overall survival and also highlights the inefficacy of hematopoietic stem cell transplantation for patients with advanced disease.