From H1N1 to COVID-19 : what we have seen in children with hemoglobinopathies

This work aimed to better understand the impact of pandemics of respiratory viruses on children with hemoglobinopathies through a comprehensive review of the literature. MEDLINE, SCIELO, LILACS, and PUBMED were used as data sources to find articles without time period restrictions. Previous observations suggest that patients with hemoglobinopathies are a group especially susceptible to the complications of viral respiratory infections, with greater morbidity and mortality related to them. Within this context, this review found that, during the 2009 H1N1 pandemic, the risk of hospitalization in children and adults increased, especially in patients with a history of complications such as acute chest syndrome. In addition, the Coronavirus Disease 2019 (COVID-19) pandemic appears to have less repercussion among children with hemoglobinopathies compared to adults, similar to what is seen in the general population. In the H1N1 pandemic, patients with hemoglobinopathies behaved as a group more susceptible to complications, with increased morbidity and mortality. However, for COVID-19, the existing data to date on these patients do not show the same clinical impact. Thus, although these children deserve attention in case of infection due to their potential risks, they seem to have a favorable evolution

Are there regional variations in the presentation of childhood leukemia?

Introduction: Treatment of childhood acute lymphoblastic leukemia (ALL) is basedon risk stratification. This study aimed to assess the agreement between risk groupclassifications in the different childhood ALL treatment protocols used in a referralhospital in southern Brazil.Methods: We retrospectively reviewed the medical records of patients aged 1 to18 years with B-cell ALL treated at a hospital from January 2013 to April 2017. Agreementbetween risk classifications was assessed by the kappa coefficient.Results: Seventy-five patients were analyzed. There was poor agreement betweenrisk stratification by GBTLI 2009 and BFM 95 protocols (kappa=0.22; p = 0.003) andby GBTLI 2009 and IC-BFM 2002 protocols (kappa=0.24; p = 0.002). Risk groupdistribution was 13.3% for low risk, 32.0% for intermediate risk, and 54.7% for highrisk based on stratification by the GBTLI 2009 protocol, and 28.0% for low risk, 42.7%for intermediate risk, and 29.3% for high risk based on stratification by the IC-BFM2002 protocol. Overall survival was 68.6%.Conclusion: This study provides numerous points to ponder about the treatmentof leukemia in Brazil. The percentage of patients classified as high risk in oursample was higher than that reported in the international literature. This difference,however, had no impact on overall survival, which was shorter than that reportedin the international literature

Are there regional variations in the presentation of childhood leukemia?

Introduction: Treatment of childhood acute lymphoblastic leukemia (ALL) is based on risk stratification. This study aimed to assess the agreement between risk group classifications in the different childhood ALL treatment protocols used in a referral hospital in southern Brazil. Methods: We retrospectively reviewed the medical records of patients aged 1 to 18 years with B-cell ALL treated at a hospital from January 2013 to April 2017. Agreement between risk classifications was assessed by the kappa coefficient. Results: Seventy-five patients were analyzed. There was poor agreement between risk stratification by GBTLI 2009 and BFM 95 protocols (kappa=0.22; p = 0.003) and by GBTLI 2009 and IC-BFM 2002 protocols (kappa=0.24; p = 0.002). Risk group distribution was 13.3% for low risk, 32.0% for intermediate risk, and 54.7% for high risk based on stratification by the GBTLI 2009 protocol, and 28.0% for low risk, 42.7% for intermediate risk, and 29.3% for high risk based on stratification by the IC-BFM 2002 protocol. Overall survival was 68.6%. Conclusion: This study provides numerous points to ponder about the treatment of leukemia in Brazil. The percentage of patients classified as high risk in our sample was higher than that reported in the international literature. This difference, however, had no impact on overall survival, which was shorter than that reported in the international literature

Allergic reactions to asparaginase : retrospective cohort study in pediatric patients with acute lymphoid leukemia

Introduction To assess the frequency of allergic reactions to asparaginase (ASP) and possible risk factors for reactions in a cohort of pediatric patients. Method The study was performed based on retrospective data from patients under acute lymphoid leukemia treatment in a general university hospital located in southern Brazil. Information on patients who used ASP from 2010 to 2017 was collected. Allergic reactions were identified in electronic medical records. Results Among the 98 patients included in the study, 16 (16.3 %) experienced an allergic reaction to native l-asparaginase (L-ASP). Of the 22 patients (22.4 %) that received only intravenous (IV) administration of l-ASP, 10 (62.5 %) had allergic reactions, while 48 patients (49 %) received intramuscular (IM) administration and 28 (28.6 %) received IV and IM administrations. The occurrence of allergic reactions differed between the groups (p <  0.001), and IV administration was associated with allergic reactions. Association was also observed between the severity of the reaction and the route of administration, with the IM route associated with grade 2 and IV route associated with grade 3. Occurrence of allergic reactions was higher when the commercial formulation of l-ASP, Leuginase®, was used (p =  0.0009 in the analysis per patient and p =  0.0003 in the analysis per administration). Conclusions The IV administration and commercial Leuginase® presentation were associated with more allergic reactions in the study population, which corroborates the findings in the literature. The IV route was also associated with higher severity of reactions in the present study

Survival of patients submitted to allogeneic bone marrow transplantation

Introdução: O transplante de células-tronco hematopoiéticas (TCTH) alogênico é um procedimento que oferece um potencial de cura para doenças hematológicas malignas e benignas. O benefício da técnica está especialmente relacionado ao aumento da sobrevida em pacientes com doadores HLA-compatíveis em cujos casos o tratamento quimioterápico mostrouse insuficiente ou ineficaz. Objetivos: Analisar a sobrevida de pacientes que receberam TCTH alogênico aparentado no Serviço de Hematologia Clínica e Transplante de Medula Óssea (SHCTMO) do Hospital de Clínicas de Porto Alegre (HCPA). Métodos: Estudo de coorte prospectiva com análise de sobrevida de pacientes transplantados entre 1994 e 2003. Resultados: Foram analisados 133 pacientes com idade média de 30,8±14,8 anos com um tempo médio de 26,8 meses entre o diagnóstico e o TCTH. Cinco anos após o transplante, 71 pacientes (53,4%) estavam vivos, 22 pacientes tinham leucemia mieloide aguda (LMA), 54, leucemia mieloide crônica (LMC), e seis padeciam de síndrome mielodisplásica (SMD), sendo que, em 5 anos, a sobrevida foi de 52, 50 e 33%, respectivamente. Dos 26 pacientes transplantados por anemia aplásica (AA), 66,7% tinham idade inferior a 20 anos, e 61,5% dos que tinham mais de 20 anos estavam vivos. Conclusão: Embora, no nosso estudo, o tempo médio entre o diagnóstico e o transplante tenha sido superior a 2 anos, e embora nossa análise tenha sido apenas estratificada pelo tipo da doença, independentemente do regime de condicionamento ou da fase da doença no momento do TCTH, nossos resultados são superponíveis aos descritos na literatura mundial.Background: Hematopoietic stem cell transplantation (HSCT) represents a curative alternative for malignant and benign hematological diseases. The benefits of the technique are especially related to an increase in the survival of patients with HLA-compatible hematopoietic stem cell donors when chemotherapy or clinical therapy has resulted ineffective. Objectives: To analyze the survival of patients submitted to allogeneic HSCT at the Hematology and Bone Marrow Transplant Service of Hospital de Clínicas de Porto Alegre. Methods: A prospective cohort of all patients submitted to transplantation between 1994 and 2003 was analyzed for overall survival. Results: A total of 133 patients were submitted to transplantation in the study period, with a mean age of 30.8±14.8 years; mean time elapsed between diagnosis and transplant was 26.8 months. Five years after the procedure, 71 patients (53.4%) were alive, 22 patients had acute and 54 had chronic myeloid leukemia, and six patients presented myelodysplastic syndrome; the 5 year overall survival was 52, 50, and 33%, respectively. Of the 26 patients transplanted for aplastic anemia, 66.7% had 20 or less years of age, and 61.5% of the patients older than 20 years were alive. Conclusion: Although the mean time elapsed between diagnosis and transplantation was over 2 years and although our results were stratified by type of disease only, the findings herein reported are similar to those found in the literature, independently of conditioning regimen or disease stage at the time of transplant

Risk factors for severe COVID-19 infection in Brazilian children

The aim of this study was to describe the epidemiological characteristics and clinical outcome of children hospitalized with COVID-19 and identify the risk factors for severe disease. All hospital admissions of pediatric patients between March and December 2020 in the southern region of Brazil were reviewed and the patients positive for RT-PCR for SARS-CoV-2 were identified. This region encompasses a population of over 2.8 million children and adolescents. Data were extracted from a national database that includes all cases of severe acute respiratory syndrome requiring hospitalization in Brazil. A total of 288 hospitalizations (51.3% female) with a median age of 3 years (interquartile range 0-12 years) were identified. Of these, 38.9% had chronic medical conditions, 55.6% required some form of supplementary oxygen, and 30.2% were admitted to an intensive care unit. There were 17 deaths (5.9%) related to COVID-19. Age less than 30 days was significantly associated with increased odds of critical illness (OR 9.52, 95% CI 3.01-30.08), as well as the presence of one chronic condition (OR 5.08 95% CI 2.78-9.33) or two or more chronic conditions (OR 6.60, 95% CI 3.17-13.74). Conclusion: Age under 30 days old and presence of chronic conditions were strongly associated with unfavorable outcomes in Brazilian children with SARS-CoV-2 infection. These findings could help local public health authorities to develop specific policies to protect this more vulnerable group of children

Influence of minimal residual disease by multiparametric flow cytometry at day 15 of induction in risk stratification of children with B-cell acute lymphoblastic leukemia treated at a referral hospital in southern Brazil

Background: The minimal residual disease (MRD) is the most important prognostic factor for acute lymphoblastic leukemia (ALL) in children. This study aimed to investigate the influence of detecting the MRD by the multiparametric flow cytometry (MFC) at day 15 (D15) of the induction on the analysis of the risk group classifications of the different childhood ALL treatment protocols used in a referral hospital in southern Brazil. Method: We retrospectively reviewed the medical records of patients with B-cell ALL, aged 1 to 18 years, treated at a hospital from January 2013 to April 2017. Main results: Seventy-five patients were analyzed. Regarding the MRD by the MFC at D15, the analyses showed statistical significance when the MRD was grouped into three categories, 10%, with the following distribution: 30.7%, 52.0%, and 17.3%, respectively. There was a significant association between D15 MRD-MFC 10% and the likelihood of dying or relapsing. The cumulative hazard ratio for the relapse of patients with D15 MRD-MFC 10% was 19.2%, 59.8%, and 80.1%, respectively. Conclusion: Our analysis suggests D15 MRD-MFC < 0.1% as a cut-off point for patients with more favorable outcomes and that the MRD at D15 in risk classifications is particularly useful for the stratification of patients with a more favorable prognosis