Trasplante de médula ósea haploidéntico en un paciente pediátrico con síndrome de Wiskott-Aldrich. A propósito de un caso / Haploidentical bone marrow transplantation in a pediatric patient with Wiskott-Aldrich syndrome. A case report

El síndrome de Wiskott-Aldrich es un error innato de la inmunidad de herencia ligada al cromosoma X, producido por variantes en el gen que codifica la proteína del síndrome de Wiskott-Aldrich (WASp). Reportamos el caso clínico de un paciente de 18 meses con diagnóstico de Wiskott-Aldrich que no presentaba donante antígeno leucocitario humano (HLA) idéntico y recibió un trasplante de células progenitoras hematopoyéticas (TCPH) con donante familiar haploidéntico. La profilaxis para enfermedad de injerto contra huésped incluyó ciclofosfamida (PT-Cy). El quimerismo del día +30 fue 100 % del donante y la evaluación postrasplante de la expresión de la proteína WAS fue normal. Actualmente, a 32 meses del trasplante, presenta reconstitución hematológica e inmunológica y quimerismo completo sin evidencia de enfermedad injerto contra huésped. El TCPH haploidéntico con PT-Cy se mostró factible y seguro en este caso de síndrome de WiskottAldrich en el que no se disponía de un donante HLA idéntico.

Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp). Here, we report the clinical case of an 18-month-old boy diagnosed with Wiskott-Aldrich syndrome, who did not have an HLA-matched related or unrelated donor and was treated successfully with a hematopoietic stem cell transplant (HSCT) from a haploidentical family donor. Graft-versus-host disease (GvHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy). At day +30, the peripheral blood-nucleated cell chimerism was 100% and the WAS protein had a normal expression. Currently, at month 32 post-transplant, the patient has hematological and immune reconstitution and complete donor chimerism without evidence of GvHD. HSCT with PT-Cy was a feasible and safe option for this patient with WAS, in which an HLA matched donor was not available.

Prevalence and clinical consequences of Hepatitis C virus infection in patients undergoing hematopoietic stem cell transplantation

ABSTRACT Hepatitis C virus (HCV) infection is a significant cause of morbidity and mortality among hematopoietic stem cell transplant (HCT) recipients. In Brazil, its occurrence in HCT recipients remains undetermined. We now report on HCV prevalence in HCT recipients and its clinical consequences. The medical records of all HCT recipients seen at Hospital das Clinicas, Sao Paulo University Medical School, from January 2010 to January 2020 were reviewed to determine HCV serostatus. A retrospective analysis of medical charts was undertaken on all seropositive cases to determine HCV genotype, presence of liver fibrosis, co-infections with other viruses, previous treatments, and clinical evolution of liver pathology after HCT. Of the 1,293 HCT recipients included in the study, seven (0.54%) were HCV antibody-positive and five (0.39%) were also viremic for HCV-RNA. Four of these individuals had moderate to severe liver fibrosis (METAVIR F2/F3) and one was cirrhotic. Two of the viremic patients developed acute liver dysfunction following transplantation. All patients had their acute episode of liver dysfunction resolved with no further complications. Four of the viremic patients were treated for HCV infection with direct acting agents (DAA). Information regarding HCV treatment was lacking for one of the viremic HCV patients due to loss of follow up. Sustained anti-virologic responses were observed in three cases after the use of DAA. The detection of HCV in hematological adults undergoing HCT and its successful treatment with DAA highlight the necessity of testing for HCV both prior to and following transplantation.

COVID-19 in hematopoietic stem cell transplant recipients during three years of the pandemic: a multicenter study in Brazil

ABSTRACT Hematopoietic stem cell transplant (HSCT) recipients are at -increased risk for severe COVID-19. The aim of this study was to evaluate the burden of COVID-19 in a cohort of HSCT recipients. This retrospective study evaluated a cohort of adult hospitalized HSCT recipients diagnosed with COVID-19 in two large hospitals in São Paulo, Brazil post-HSCT, from January 2020 to June 2022. The primary outcome was all-cause mortality. Of 49 cases, 63.2% were male with a median age of 47 years. Allogeneic-HSCT (51.2%) and autologous-HSCT (48.9%) patients were included. The median time from HSCT to COVID-19 diagnosis was 398 days (IQR: 1211-134), with 22 (44.8%) cases occurring within 12 months of transplantation. Most cases occurred during the first year of the pandemic, in non-vaccinated patients (n=35; 71.4%). Most patients developed severe (24.4%) or critical (40.8%) disease; 67.3% received some medication for COVID-19, primarily corticosteroids (53.0%). The probable invasive aspergillosis prevalence was 10.2%. All-cause mortality was 40.8%, 51.4% in non-vaccinated patients and 14.2% in patients who received at least one dose of the vaccine. In the multiple regression analyses, the variables mechanical ventilation (OR: 101.01; 95% CI: 8.205 - 1,242.93; p = 0.003) and chest CT involvement at diagnosis ≥50% (OR: 26.61; 95% CI: 1.06 - 664.26; p = 0.04) remained associated with all-cause mortality. Thus, HSCT recipients with COVID-19 experienced high mortality, highlighting the need for full vaccination and infection prevention measures.

Research advances in hepatic sinusoidal obstruction syndrome / 临床肝胆病杂志

Hepatic sinusoidal obstruction syndrome （HSOS）， also known as hepatic veno-occlusive disease， is hepatic vascular disease of hepatic sinusoidal obstruction and hepatic venular occlusion and fibrosis due to various causes. This article systematically elaborates on the research advances in HSOS from the aspects of understanding and naming， etiology and pathogenesis， clinical manifestation， diagnosis and differential diagnosis， prevention， and treatment. HSOS can occur in patients receiving bone marrow hematopoietic stem cell transplantation， radiotherapy/chemotherapy， and medication containing pyrrolidine alkaloids， and the common clinical manifestations of HSOS include abdominal distension， distending pain in the liver area， ascites， jaundice， and hepatomegaly. The diagnostic criteria for HSOS vary with etiology， and it needs to be differentiated from other diseases such as drug-induced liver diseases and hepatic venous outflow tract obstruction. Defibrotide and low-molecular-weight heparin have a therapeutic effect on HSOS associated with hematopoietic stem cells and pyrrolidine alkaloids， respectively， and there are currently no effective drugs for HSOS caused by oxaliplatin chemotherapy.

Calidad de vida y toxicidad financiera de los receptores de trasplantes de células madre hematopoyéticas en COVID-19 / Quality of life and financial toxicity of hematopoietic stem cell transplant recipients in COVID-19 / Qualidade de vida e toxicidade financeira dos transplantados de células-tronco hematopoéticas na COVID-19

Objetivo: evaluar y correlacionar la calidad de vida y la toxicidad financiera de pacientes adultos sometidos a trasplante de células madre hematopoyéticas durante el período de la pandemia de COVID-19. Método: estudio observacional, analítico, realizado con 35 pacientes en un hospital de referencia para trasplante en Latinoamérica. Para la recolección de datos, se utilizaron los cuestionarios Functional Assessment Cancer Therapy Bone Marrow Transplantation y el COmprehensive Score for financial Toxicity. Para el análisis de los datos se utilizaron las pruebas de correlación de Spearman y Mann-Whitney. Resultados: la calidad de vida general durante la COVID-19 mostró un puntaje bajo (67,09/108) con mayor deterioro en el bienestar funcional (14,47/28), bienestar social (16,76/28) y preocupaciones adicionales (23,41/40). Los promedios del grupo alogénico fueron inferiores a los del grupo autólogo en todos los dominios, presentando diferencia significativa en relación a preocupaciones adicionales (p=0,01) y en el índice de evaluación del tratamiento (p=0,04). Se consideró que la toxicidad financiera tenía un impacto leve (22.11/44). Se observó una relación, aunque no significativa, entre la calidad de vida y la toxicidad financiera (p=0,051). Conclusión: la calidad de vida de la muestra fue baja; existe una correlación entre la calidad de vida y la toxicidad financiera, aunque no significativa. Cuanto mayor es la toxicidad financiera, menor es la calidad de vida.

Objective: to evaluate and correlate the quality of life and financial toxicity of adult patients undergoing hematopoietic stem cell transplantation during the COVID-19 pandemic. Method: observational, analytical study, carried out with 35 patients in a reference hospital for transplantation in Latin America. For data collection, the Functional Assessment Cancer Therapy Bone Marrow Transplantation and COmprehensive Score for Financial Toxicity questionnaires were used. Spearman and Mann-Whitney correlation tests were used for data analysis. Results: general quality of life during COVID-19 had a low score (67.09/108) with greater impairment in functional well-being (14.47/28), social well-being (16.76/28) and additional concerns (23.41/40). The means of the allogeneic group were lower than those of the autologous group in all domains, showing a significant difference in relation to additional concerns (p=0.01) and in the treatment evaluation index (p=0.04). Financial toxicity was considered to have a slight impact (22.11/44). There was a relationship, albeit not significant, between quality of life and financial toxicity (p=0.051). Conclusion: the quality of life of the sample was low; there is a correlation between quality of life and financial toxicity, although not significant. The higher the financial toxicity, the lower the quality of life.

Objetivo: avaliar e correlacionar a qualidade de vida e a toxicidade financeira dos pacientes adultos submetidos ao transplante de células-tronco hematopoéticas no período da pandemia de COVID-19. Método: estudo observacional, analítico, realizado com 35 pacientes em um hospital de referência para o transplante na América Latina. Para coleta de dados, utilizaram-se os questionários Functional Assessment Cancer Therapy Bone Marrow Transplantation e COmprehensive Score for financial Toxicity. Na análise dos dados empregaram-se os testes de correlação de Spearman e Mann-Whitney. Resultados: a qualidade de vida geral, durante a COVID-19, apresentou baixo escore (67,09/108), com maior comprometimento nas funções bem-estar funcional (14,47/28), social (16,76/28) e preocupações adicionais (23,41/40). As médias do grupo alogênico foram inferiores às do autólogo em todos os domínios, apresentando diferença significativa em relação às preocupações adicionais (p=0,01) e ao índice de avaliação do tratamento (p=0,04). A toxicidade financeira foi considerada de impacto leve (22,11/44). Observou-se relação, ainda que não significativa, entre a qualidade de vida e a toxicidade financeira (p=0,051). Conclusão: a qualidade de vida da amostra foi baixa, logo há uma correlação entre qualidade de vida e a toxicidade financeira, embora não significativa. Quanto maior a toxicidade financeira, menor a qualidade de vida.

Utilidad del lavado broncoalveolar para la detección microbiológica, y factores asociados, en receptores de trasplante de precursores hematopoyéticos: estudio de cohorte retrospectivo

Introducción: Las opacidades pulmonares en receptores de trasplante de precursores hematopoyéticos (TPH) representan un desafío diagnóstico y son una causa de morbimortalidad. Existen grandes discrepancias con respecto a la sensibilidad diagnóstica del lavado broncoalveolar (LBA), sus complicaciones, y los factores asociados a la identificación microbiológica. Objetivo: Conocer la utilidad del estudio microbiológico del LBA en el diagnóstico, modificación de la conducta médica y estimar las complicaciones y mortalidad asociada al procedimiento, en receptores de TPH con opacidades pulmonares. Pacientes y Métodos: Estudio de cohorte, retrospectivo, en adultos receptores de TPH a los que se les realizó una broncoscopía con LBA por presentar opacidades pulmonares, en el Hospital Italiano de Buenos Aires entre el 01/01/2011 y el 31/12/2020. Resultados: De los 189 procedimientos analizados, en 79 se logró un hallazgo microbiológico (41,8%) y 122 permitieron modificar la conducta médica (64,6%). En 11 casos se observaron complicaciones graves dentro de las 12 horas (5,8%) de efectuado el LBA. La mortalidad intrahospitalaria fue de 16,8% (N = 21/125). El valor de neutrófilos en sangre previo al LBA (p = 0,037) y la presencia de nódulos pulmonares como lesión tomográfica predominante (p = 0,029) se asociaron independientemente al hallazgo microbiològico global. Conclusiones: Nuestra investigación apoya la realización del LBA como herramienta diagnóstica en pacientes que reciben un TPH y presentan opacidades pulmonares.

Background: Lung opacities are a cause of morbimortality in bone marrow transplant patients, and represent a diagnostic challenge. There are large discrepancies regarding the diagnostic sensitivity of bronchoalveolar lavage (BAL), its complications, and the factors associated with microbiological detection. Aim: To know the usefulness of the microbiological study of BAL in the diagnosis, in the modification in medical behavior and to estimate the complications and associated mortality of this diagnostic procedure in patients transplanted with hematopoietic progenitor cells with pulmonary opacities. Methods: Retrospective cohort study in bone marrow transplant adult patients who underwent bronchoscopy with BAL due to lung opacities at Hospital Italiano de Buenos Aires between 01/01/2011 and 12/31/2020. Results: Of the 189 BAL analyzed, 79 presented a microbiological detection (41.8%) and 122 allowed to modify the medical behavior (64.6%). Severe complications were observed within 12 hours after the procedure in11 cases (5.8%). In-hospital mortality was 16,8% (N = 21/125). The value of blood neutrophils prior to bronchoalveolar lavage (p = 0.037) and the presence of pulmonary nodules as the predominant tomographic lesion (p = 0.029) were independently associated with global microbiological detection. Conclusion: Our research supports the performance of BAL as a diagnostic tool in bone marrow transplant patients with lung opacities.

Clinical characterization and outcomes of a cohort of colombian patients with AL Amyloidosis

Background: Amyloid light chain (AL) amyloidosis is characterized by amyloid fibril deposition derived from monoclonal immunoglobulin light chains, resulting in multiorgan dysfunction. Limited data exist on the clinical features of AL amyloidosis. Objective: This study aims to describe the clinical characteristics, treatments, and outcomes in Colombian patients with AL amyloidosis. Methods: A retrospective descriptive study was conducted at three high-complexity centers in Medellín, Colombia. Adults with AL amyloidosis diagnosed between 2012 and 2022 were included. Clinical, laboratory, histological, treatment, and survival data were analyzed. Results: The study included 63 patients. Renal involvement was most prevalent (66%), followed by cardiac involvement (61%). Multiorgan involvement occurred in 61% of patients. Amyloid deposition was most commonly detected in renal biopsy (40%). Bortezomib-based therapy was used in 68%, and 23.8% received high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HDCT-ASCT). Hematological response was observed in 95% of patients with available data. Cardiac and renal organ responses were 15% and 14%, respectively. Median overall survival was 45.1 months (95% CI: 22.2-63.8). In multivariate analysis, cardiac involvement was significantly associated with inferior overall survival (HR 3.27; 95% CI: 1.23-8.73; p=0.018), HDCT-ASCT had a non-significant trend towards improved overall survival (HR 0.25; 95% CI: 0.06-1.09; p=0.065). Conclusions: In this study of Colombian patients with AL amyloidosis, renal involvement was more frequent than cardiac involvement. Overall survival and multiorgan involvement were consistent with data from other regions of the world. Multivariate analysis identified cardiac involvement and HDCT-AHCT as possible prognostic factors.

Antecedentes: La amiloidosis por amiloide de cadenas ligeras (AL) se caracteriza por el depósito de fibrillas amiloides derivadas de cadenas ligeras de inmunoglobulinas monoclonales, lo que resulta en disfunción multiorgánica. Existen datos limitados sobre las características clínicas de la amiloidosis AL. Objetivo: Este estudio tiene como objetivo describir las características clínicas, tratamientos y desenlaces en pacientes colombianos con amiloidosis AL. Métodos: Se llevó a cabo un estudio descriptivo retrospectivo en tres centros de alta complejidad en Medellín, Colombia. Se incluyeron adultos con diagnóstico de amiloidosis AL entre 2012 y 2022. Se analizaron datos clínicos, de laboratorio, histológicos, de tratamiento y de supervivencia. Resultados: El estudio incluyó 63 pacientes. La afectación renal fue más prevalente (66%), seguida de la afectación cardíaca (61%). El 61% de los pacientes presentaron afectación multiorgánica. El depósito amiloide se detectó con mayor frecuencia en la biopsia renal (40%). El tratamiento basado en bortezomib se utilizó en el 68%, y el 23.8% recibió altas dosis de quimioterapia con trasplante autólogo de progenitores hematopoyéticos (ADQT-TAPH). Se observó respuesta hematológica en el 95% de los pacientes con datos disponibles. La respuesta de órgano cardíaca y renal fue del 15% y 14%, respectivamente. La mediana de la supervivencia global fue de 45.1 meses (IC del 95%: 22.2-63.8). En el análisis multivariado, la afectación cardíaca se asoció significativamente con una supervivencia global inferior (HR 3.27; IC del 95%: 1.23-8.73; p=0.018), ADQT-TAPH mostró una tendencia no significativa hacia una mejora en la supervivencia global (HR 0.25; IC 95%: 0.06-1.09; p=0.065). Conclusiones: En este estudio de pacientes colombianos con amiloidosis AL, la afectación renal fue más frecuente que la afectación cardíaca. La supervivencia global y la afectación multiorgánica fueron consistentes con datos de otras regiones del mundo. El análisis multivariado identificó la afectación cardíaca y ADQT-TAPH como posibles factores pronósticos.

Dental consensus on HSCT - Part II: dental Care during HSCT

ABSTRACT During the state of immune vulnerability in hematopoietic stem cell transplantation (HSCT), the patient has an increased risk of developing a vast number of complications, including severe problems in the oral cavity. These situations require professional oral care to act in the diagnosis and treatment of these conditions, as well as to develop prevention protocols to minimize patient's complications. Oral mucositis, opportunistic infections, bleeding, specific microbiota, taste, and salivary alterations are complications that can occur during HSCT and interfere with various aspects, such as pain control, oral intake, nutrition, bacteremia and sepsis, days of hospitalization and morbidity. Several guidelines have been published to address the role of professional oral care during the HSCT, we describe a consensus regarding these recommendations.

Brazilian dental consensus on dental management in hematopoietic stem cell transplantation−Part I−pre-HSCT

ABSTRACT The oral involvement in the Hematopoietic Stem Cell Transplantation is well described in the literature. The goal of the dental treatment and management of the oral lesions related to the HSCT is to reduce the harm caused by preexisting oral infection or even the worsening of oral acute/chronic GVHD and late effects. The aim of this guideline was to discuss the dental management of patients subjected to HSCT, considering three phases of the HSCT: pre-HSCT, acute phase, and late phase. The literature published from 2010 to 2020 was reviewed in order to identify dental interventions in this patient population. The selected papers were divided into three groups: pre-HSCT, acute and late, and were reviewed by the SBTMO Dental Committee's members. When necessary, an expertise opinion was considered for better translating the guideline recommendations to our population dental characteristics. This manuscript focused on the pre-HSCT dental management. The objective of the pre-HSCT dental management is to identify possible dental situations that On behalf of the Dental Committee of the Brazilian Society of Gene Therapy and Bone Marrow Transplantation (SBTMO) can worsening during the acute phase after the HSCT. Each guideline recommendations were made considering the Dentistry Specialties. The clinical consensus on dental management prior to HSCT provides professional health caregivers with clinical setting-specific information to help with the management of dental problems in patients to be subjected to HSCT.

Need for hemodialysis in patients undergoing hematopoietic stem cell transplantation: risk factors and survival in a retrospective cohort

ABSTRACT Introduction: Allogeneic Hematopoietic Stem Cell Transplantation (allo-HSCT) patients are exposed to acute and chronic nephrotoxic events (drugs, hypotension, infections, and microangiopathy). The need for hemodialysis (HD) may be associated with high mortality rates. However, the risk factors and clinical impact of HD are poorly understood. Aim: To analyze survival and risk factors associated with HD in allo-HSCT Patients and methods: single-center cohort study 185 (34 HD cases versus 151 controls) consecutive adult allo-HSCT patients from 2007-2019. We performed univariate statistical analysis, then logistic regression and competing risk regression were used to multivariate analysis. Survival was analyzed by Kaplan-Meier and Cox proportional-hazards models. Results: The one-year HD cumulative incidence was 17.6%. Univariate analysis revealed that HD was significantly associated with male gender, age (p 0.056), haploidentical donor, grade II-IV acute GVHD, polymyxin B, amikacin, cidofovir, microangiopathy, septic shock (norepinephrine use) and steroid exposure. The median days of glycopeptides exposure (teicoplanin/vancomycin) was 16 (HD) versus 10 (no HD) (p 0.088). In multivariate analysis, we found: norepinephrine (hazard ratio, HR:3.3; 95% confidence interval, 95%CI:1.2-8.9; p 0.024), cidofovir drug (HR:11.0; 95%CI:4.6 - 26.0; p < 0.001), haploidentical HSCT (HR:1.94; 95%CI:0.81-4.65; p 0.14) and Age (HR:1.01; 95%CI: 0.99-1.03; p 0.18). The HD group had higher mortality rate (HR:6.68; 95% CI: 4.1-10.9; p < 0.001). Conclusion: HD was associated with decreased survival in allo-HSCT. Carefully use of nephrotoxic drugs and improving immune reconstitution could reduce severe infections (shock) and patients requiring cidofovir, which taken together may result in lower rates of HD, therefore improving survival.

Dentistry consensus on HSCT - Part III: Special topics - Dentistry on HSCT

ABSTRACT Patients undergoing hematopoietic stem cell transplantation (HSCT) might present acute and late toxicities and the oral tissues are frequently affected. With the survival increasing, patients show late and long-term morbidities, and there is an important association between the general and the oral health. The first and second parts of this Consensus have showed the importance of the adequacy of oral health in the pre-HSCT, and the main alterations and oral care during the period of admission for HSCT. This third part aims to review specific themes of post-HSCT dental care, such as graft-versus-host disease (GVHD) and the pediatric patient. It also aims to review pertinent subjects, both during the HSCT period and post-HSCT, concerning quality of life, pain, cost-effectiveness, and remote care. Based on this review, it is evident the importance of the work of the dental surgeon (DS) in the follow-up and treatment of the HSCT patient, always collaborating with the whole multidisciplinary team.

One-year mortality of hematopoietic stem cell recipients admitted to an intensive care unit in a dedicated Brazilian cancer center: a retrospective cohort study

ABSTRACT BACKGROUND: Hematopoietic stem cell transplantation (HSCT) recipients requiring intensive care unit (ICU) admission early after transplantation have a poor prognosis. However, many studies have only focused on allogeneic HSCT recipients. OBJECTIVES: To describe the characteristics of HSCT recipients admitted to the ICU shortly after transplantation and assess differences in 1-year mortality between autologous and allogeneic HSCT recipients. DESIGN AND SETTING: A single-center retrospective cohort study in a cancer center in Brazil. METHODS: We included all consecutive patients who underwent HSCT less than a year before ICU admission between 2009 and 2018. We collected clinical and demographic data and assessed the 1-year mortality of all patients. The effect of allogeneic HSCT compared with autologous HSCT on 1-year mortality risk was evaluated in an unadjusted model and an adjusted Cox proportional hazard model for age and Sequential Organ Failure Assessment (SOFA) at admission. RESULTS: Of the 942 patients who underwent HSCT during the study period, 83 (8.8%) were included in the study (autologous HSCT = 57 [68.7%], allogeneic HSCT = 26 [31.3%]). At 1 year after ICU admission, 21 (36.8%) and 18 (69.2%) patients who underwent autologous and allogeneic HSCT, respectively, had died. Allogeneic HSCT was associated with increased 1-year mortality (unadjusted hazard ratio, HR = 2.79 [confidence interval, CI, 95%, 1.48-5.26]; adjusted HR = 2.62 [CI 95%, 1.29-5.31]). CONCLUSION: Allogeneic HSCT recipients admitted to the ICU had higher short- and long-term mortality rates than autologous HSCT recipients, even after adjusting for age and severity at ICU admission.

Long-term kidney outcomes in children after allogeneic hematopoietic stem cell transplantation assessed with estimated glomerular filtration rate equations, creatinine levels, and cystatin C levels / Desfechos renais a longo prazo em crianças após transplante alogênico de células-tronco hematopoiéticas avaliados com equações de taxa de filtração glomerular estimada, níveis de creatinina e níveis de cistatina C

Abstract Background and objective: With the widespread use of allogeneic hematopoietic stem cell transplantation (allo-HSCT), long-term complications have come to the fore. The aim of this study was to determine the prevalence and risk factors of chronic kidney disease (CKD) developing in the long term in patients who underwent allo-HSCT in childhood and also to investigate the superiority of eGFR formulas. Methods: The present study evaluated CKD in patients who underwent allo-HSCT. We analyzed the 94 children who received allo-HSCT at the Ege University in İzmir between August and November, 2019. The patients were evaluated at 2 years after transplantation. CKD was defined as a glomerular filtration rate (GFR) <90 mL/min/1.73 m2 using eGFR equations based on serum creatinine (SCr), cystatin C (CysC), and SCr plus CysC. Results: In our study, 9 (9.4%), according to Bedside Schwartz, 59 (76.6%), according to CKiD-eGFR-CysC, and 20 (26%) patients, according to CKiD-eGFR-SCr-CysC equations were identified with CKD. In cases identifies as CKD according to CysC, early development of acute kidney injury (AKI), post-transplant cytomegalovirus (CMV) reactivation and being >120 months during transplantation were found to be associated with the development of CKD. Conclusion: We may be delayed in detecting CKD by calculating SCr-based formulas in allo-HSCT cases, which is a patient group where early diagnosis and treatment of CKD is very important.

Resumo Antecedentes e objetivo: Com o uso generalizado do transplante alogênico de células-tronco hematopoiéticas (TCTH-alo), as complicações a longo prazo tornaram-se evidentes. O objetivo deste estudo foi determinar a prevalência e os fatores de risco do desenvolvimento de doença renal crônica (DRC) a longo prazo em pacientes submetidos a TCTH-alo na infância, e também investigar a superioridade das fórmulas de TFGe. Métodos: O presente estudo avaliou a DRC em pacientes que foram submetidos ao TCTH-alo. Analisamos as 94 crianças que receberam TCTH-alo na Universidade Ege em İzmir entre Agosto e Novembro de 2019. Os pacientes foram avaliados aos 2 anos após o transplante. A DRC foi definida como uma taxa de filtração glomerular (TFG) <90 mL/min/1,73 m2 usando equações de TFGe baseadas em creatinina sérica (CrS), cistatina C (CisC), e CrS mais CisC. Resultados: Em nosso estudo, 9 pacientes (9,4%), de acordo com a equação de Schwartz (à beira do leito), 59 (76,6%), de acordo com a equação DRC-TFGe-CisC, e 20 (26%) pacientes, de acordo com a equação DRC-TFGe-CrS-CisC, foram classificados com DRC. Quando a TFG é avaliada pela CisC, verificamos que o desenvolvimento precoce de lesão renal aguda (LRA), a reativação do citomegalovírus (CMV) pós-transplante e ter >120 meses durante o transplante foram associados ao desenvolvimento de DRC. Conclusão: Pode haver atraso na detecção da DRC quando usamos fórmulas baseadas em CrS em casos de TCTH-alo, que é um grupo de pacientes onde o diagnóstico e tratamento precoces da DRC são muito importantes.

Effectiveness of the peripherally inserted central catheter for hematopoietic stem cell transplantation: a systematic review protocol

OBJECTIVE: to assess the effectiveness and safety of the peripherally inserted central catheter for hematopoietic stem cell transplantation. METHOD: this review will follow the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses, and the search steps will be presented through the flow diagram. The search strategy aims to locate both published and unpublished studies. No time or language restrictions will be applied. The review will consider experimental and observational studies that include adult and pediatric patients undergoing hematopoietic stem cell transplantation. Patients using peripherally inserted central catheters will be compared with those using other central catheters.

Self-care guidelines for patients in the post-hematopoietic stem cell transplantation period: a scoping review / Orientaciones para el autocuidado de pacientes en el post-trasplante de células madre hematopoyéticas: revisión de alcance / Orientações para o autocuidado de pacientes no pós-transplante de células-tronco hematopoéticas: revisão de escopo

ABSTRACT Objective: To map the evidence on self-care guidelines for patients in the post-hematopoietic stem cell transplantation (HSCT) period. Method: Scoping review supported by Joanna Briggs Institute recommendations, with searches conducted between March and April 2022 in national and international databases and repositories of theses and dissertations. Results: Of the 11 studies that composed the final sample, the guidelines had a social and personal aspect, as post-transplant patients need to follow numerous essential recommendations for the prevention of infections and complications for successful treatment and improved quality of life. Conclusion: Knowing the self-care guidelines that must be performed by post-HSCT patients is fundamental for the nursing team to provide the necessary information for care outside the controlled environment of the hospital, in addition to minimizing episodes of infection, death, and increasing the survival and quality of life of transplant recipients.

RESUMEN Objetivo: mapear las evidencias sobre las orientaciones realizadas para el autocuidado de pacientes en el post-trasplante de células madre hematopoyéticas (TCTH). Método: Scoping Review apoyada en las recomendaciones del Instituto Joanna Briggs, con búsquedas entre marzo y abril de 2022 en bases de datos y repositorios de tesis y disertaciones nacionales e internacionales. Resultados: de los 11 estudios que compusieron la muestra final, las orientaciones tenían un carácter social y personal, ya que el paciente en el post-trasplante necesita seguir numerosas recomendaciones imprescindibles para la prevención de infecciones y complicaciones para el éxito del tratamiento y la mejora de la calidad de vida. Conclusión: Conocer las orientaciones para el autocuidado que deben ser realizadas por pacientes en el post-TCTH es fundamental para que el equipo de enfermería proporcione la información necesaria para los cuidados fuera del contexto controlado del ambiente hospitalario, además de minimizar los episodios de infección, muerte y aumentar la sobrevida y calidad de vida de los transplantados.

RESUMO Objetivo: mapear as evidências sobre as orientações realizadas para o autocuidado de pacientes no pós-transplante de células-tronco hematopoéticas (TCTH). Método: Scoping Review apoiada nas recomendações do Joanna Briggs Institute, com buscas entre março e abril de 2022 em bases de dados e repositórios de teses e dissertações nacionais e internacionais. Resultados: dos 11 estudos que compuseram a amostra final, as orientações tinham cunho social e pessoal, visto que o paciente do pós-transplante precisa seguir inúmeras recomendações imprescindíveis para a prevenção de infecções e complicações para o êxito do tratamento e melhoria da qualidade de vida. Conclusão: Conhecer as orientações para o autocuidado que devem ser realizadas por pacientes no pós-TCTH é fundamental para que a equipe de Enfermagem forneça as informações necessárias para os cuidados fora do contexto controlado do ambiente hospitalar, além de minimizar os episódios de infecção, morte e aumentar a sobrevida e qualidade de vida dos transplantados.

Perfil de liberación de biomarcadores miocárdicos en pacientes con enfermedades hemato-oncológicas que reciben trasplante de progenitores hematopoyéticos / Profile or myocardial biomarkers release in patients with hemato-oncological diseases who receive hematopoietic stem cell transplantation / Perfil de liberação de biomarcadores miocárdicos em pacientes com doenças hemato-oncológicas que recebem transplante de células-tronco hematopoéticas

Introducción: las enfermedades cardiovasculares (CV) son la primera causa de muerte en quienes sobreviven al cáncer. Aunque el trasplante de progenitores hematopoyéticos (TPH) se asocia con grados variables de cardiotoxicidad, estas complicaciones han sido escasamente caracterizadas. Objetivo: analizar el perfil de liberación de biomarcadores miocárdicos como potenciales indicadores subclínicos de cardiotoxicidad en pacientes sometidos a TPH. Material y método: estudio descriptivo, analítico, prospectivo transversal y unicéntrico, reclutando pacientes derivados a la policlínica de cardio-oncología, con indicación de TPH en octubre de 2018-marzo de 2020. Se realizaron controles clínicos, ECG, bioquímicos (troponina I TnI y péptido natriurético del tipo BBNP) e imagenológicos según algoritmo de seguimiento. Las variables discretas se presentan como n (%) y las continuas mediante media ± DE o mediana RIQ. Los valores evolutivos de biomarcadores séricos se compararon mediante test de Friedman. La fracciónde eyección del VI (FEVI) basal se comparó con la de los 3 meses del TPH mediante test de Wilcoxon. Resultados: se incluyeron 19 pacientes, 37% mujeres, de 43,8 ± 15,7 años. No se detectaron modificaciones significativas de la FEVI en los controles evolutivos. En ningún caso se observó aumento de la TnI. Los valores de BNP aumentaron en 6 pacientes (32%), con diferencias significativas al mes postrasplante (basal: 13,6 1;6,1-30,9 vs. primer mes: 38,9 16,3-120,0 pg/ml, p = 0,036); con una mayor elevación en aquellos pacientes que recibieron antimetabolitos vs. otros fármacos (basal: 13,6 1;6,1-30,9 vs. al primer mes: 67,0 ;21,3-174,9 pg/ml, p = 0,039). El aumento de BNP no se asoció con el riesgo CV. Conclusión: la liberación de BNP posterior al TPH es un fenómeno frecuente (32% de los pacientes), alcanza un máximo al mes, independientemente de la FEVI. El subgrupo de pacientes que recibió antimetabolitos presentó una mayor liberación precoz de BNP.

Introduction: cardiovascular (CV) diseases are the leading cause of death in those who survive cancer. Although hematopoietic stem cell transplantation (HSCT) is associated with diverse grades of cardiotoxicity, these complications have been poorly characterized. Objective: to analyze the release profile of myocardial biomarkers as a potential subclinical marker of cardiotoxicity in patients undergoing HSCT. Material and method: descriptive, analytical, prospective, cross-sectional, single-center study, recruiting patients referred to the cardio-oncology polyclinic, with indication for HSCT in October 2018-March 2020. Clinical, ECG, biochemical and imaging controls were performed according to the algorithm of follow-up. The evolutionary values of serum biomarkers were compared using the Friedman test. Baseline LVEF was compared with that of 3 months after HSCT using the Wilcoxon test. Results: 19 patients were included, 37% women, aged 43.8 ± 15.7 years. No changes in LVEF were detected. In no case was an increase in TnI observed. BNP values increased in 6 patients (32%), with significant differences one month after transplantation (baseline: 13.6 ;6.1-30.9 vs. first month: 38.9 ;16.3-120.0, p = 0.036), detecting a greater elevation in those patients who received antimetabolites vs. other rugs (baseline: 13.6 ;6.1-30.9 vs. at the first month: 67.0 21.3-174.0, p = 0.039). The increase in BNP was not associated with CV risk. Conclusion: BNP release after HSCT is frequent (32% of our patients), reaching a maximum at one month, regardless of LVEF. The subgroup of patients who received antimetabolites had a greater early release of BNP.

Introdução: as doenças cardiovasculares (CV) são a principal causa de morte em pessoas que sobrevivem ao câncer. Embora o transplante de células-tronco hematopoéticas (TCTH) esteja associado à diverso grado de cardiotoxicidade, essas complicações têm sido mal caracterizadas. Objetivo: analisar o perfil de liberação de biomarcadores miocárdicos como potenciais marcadores subclínicos de cardiotoxicidade em pacientes submetidos ao TCTH. Material e método: estudo descritivo, analítico, prospectivo, transversal, unicéntrico, com recrutamento de pacientes encaminhados à policlínica de cardio-oncologia, com indicação de TCTH de outubro de 2018 a março de 2020. Foram realizados controles clínicos, eletrocardiográficos, bioquímicos e de imagem de acordo com o algoritmo de acompanhamento. Os valores evolutivos dos biomarcadores séricos foram comparados pelo teste de Friedman. A FEVE basal foi comparada com a de 3 meses após o TCTH usando o teste de Wilcoxon. Resultados: foram incluídos 19 pacientes, 37% mulheres, com idade de 43,8 ± 15,7 anos. Nenhuma mudança na LVEF foi detectada. Em nenhum caso foi observado um aumento de TnI. Os valores de BNP aumentaram um mês após o transplante (linha de base: 13,6 6,1-30,9; vs. primeiro mês: 38,9 16,3-120,0, p = 0,036), se detectou uma maior elevação nos pacientes que receberam antimetabólitos vs. outros medicamentos (linha de base: 13,6 ;6,1-30,9; vs. no primeiro mês: 67,0 ;21,3-174,0;, p = 0,039). O aumento do BNP não foi associado ao risco CV. Conclusão: a liberação do BNP após o TCTH é frequente (32% de nossos pacientes), podendo chegar a no máximo um mês, independente da FEVE. O subgrupo de pacientes que recebeu antimetabólitos apresentou maior liberação precoce de BNP.

Immune reconstitution after allogenic stem cell transplantation: An observational study in pediatric patients

Abstract Introduction The immune reconstitution (IR) after the allogenic hematopoietic stem cell transplantation (allo-HSCT) is a progressive process intrinsically correlated to the therapeutic success. It is essential to understand the interfering factors in IR to prevent the HSCT-related mortality. Methods We retrospectively evaluated the clinical outcomes, absolute lymphocyte counts (ALCs) and lymphocyte subtypes at different time-points of 111 pediatric patients with allogeneic HSCT for malignant and non-malignant diseases from 2013 to 2018. Results The ALCs gradually increased on D+30, D+100, and D+180 (medians 634/μL, 1022/μL and 1541/μL, respectively). On D+100, the CD3+CD8+ achieved the highest recovery rate (68%), followed by the CD16+CD56+ (47%), CD3+CD4+ (39%) and CD19+ (8%). The adequate ALC recovery was associated with age < 8 years, bone marrow grafts, myeloablative conditioning, non-use of serotherapy and non-haploidentical donors. The ALC and CD3+CD8+ on D+100 counts were higher in patients with the cytomegalovirus infection. The CD3+CD4+ recovery was associated with an age < 8 years, a non-malignant disease and a lower incidence of acute graft-versus-host disease ≥ grade 2. Furthermore, the ALC recovery on D+100 resulted in a higher overall survival, regardless of the disease type (HR 3.65, 1.05 - 12.71, p= 0.04). Conclusion Several factors influenced the IR after the allo-HSCT. The ALC ≥ 500/μL on D+100 was a simple IR predictor of survival, easily available to resource-limited centers.

Measles, mumps and rubella vaccine 12 months after hematopoietic stem cell transplantation

ABSTRACT The measles, mumps and rubella (MMR) vaccine is usually recommended from 24 months after a hematopoietic stem cell transplant (HSCT). Some authors have demonstrated that the MMR vaccination can be safe from 12 months post-HSCT in non-immunosuppressed patients, as recommended by the Brazilian National Immunization Program/Ministry of Health, since 2006. The objectives of this study were to evaluate when patients received MMR vaccine after an HSCT in our care service and if there were reports of any side effects. We retrospectively reviewed the records of HSCT recipients who received at least one MMR dose in our care service, a quaternary teaching hospital in Sao Paulo city, Brazil, from 2017 to 2021. We identified 82 patients: 75.6% (90.1% in the autologous group and 45.1% in the allogeneic group) were vaccinated before 23 months post-transplantation. None reported side effects following the vaccination. Our data support that the MMR vaccination is safe from 12 to 23 months after HSCT.

Guidelines for the self-management of people subjected to hematopoietic stem cell transplantations / Pautas para el automanejo de personas sometidas a transplantes de células madre hematopoyéticas / Diretrizes para o autogerenciamento de pessoas submetidas ao transplante de células-tronco hematopoéticas

ABSTRACT Objective: to know meanings and demands for the self-management of home-based care for Hematopoietic Stem Cell transplant recipients and to discuss these demands with nurses, aiming at proposing guidelines. Method: a qualitative study based on the convergent care research study developed between December 2020 and August 2021 in a Hematopoietic Stem Cell Transplantation Service from Juiz de Fora - Minas Gerais, carried out in two stages: semi-structured interview and small group discussion. 17 people participated in the study: 12 transplanted individuals and five nurses. Data analysis was supported by the Iramuteq software and by the synthesis and theorization proposed by convergent care research. Results: in Stage 1, performed with transplanted patients, six classes emerged that were interpreted in four subcorpuses and pointed out the meanings and demands for self-management. In Stage 2, performed with nurses, the demands found in Stage 1 were submitted to reflection in the discussion group and ratified. Based on the findings, the guidelines to meet the health self-management demands of transplanted people were as follows: professional monitoring, adherence to the treatment, maintenance of the prescribed care measures, search for guidelines and information, implications of the diagnosis, restoration of immunity, inclusion in the job market, physical limitations, and changes in everyday habits. Conclusion: the guidelines proposed reflect the understanding of the needs experienced for an individual to adapt to the health/disease process in the post-Hematopoietic Stem Cell Transplantation phase, and express the importance of carrying out strategies that promote self-management.

RESUMEN Objetivo: conocer significados y requisitos para el automanejo de las medidas de atención domiciliaria para personas sometidas a trasplantes de Células Madre Hematopoyéticas y debatir dichos requisitos con enfermeros, con vistas a proponer pautas. Método: estudio cualitativo basado en la Investigación Convergente Asistencial y desarrollado entre diciembre de 2020 y agosto de 2021 en un Servicio de Trasplantes de Células Madre Hematopoyéticas de Juiz de Fora - Minas Gerais, siguiendo dos etapas: entrevistas semiestructuradas y grupo de discusión reducido. Participaron 17 personas: 12 pacientes trasplantados y cinco enfermeros. El análisis de los datos contó con el apoyo del software Iramuteq y de la síntesis y teorización propuesta por la Investigación Convergente Asistencial. Resultados: en la Etapa 1, realizada con pacientes trasplantados, surgieron seis clases que se interpretaron en cuatro subcuerpos y señalaron los significados y los requisitos para el automanejo. En la Etapa 2, realizada con enfermeros, los requisitos que se encontraron en la Etapa 1 se sometieron a reflexión en el grupo de discusión, para luego ser ratificados. En función de los hallazgos, las pautas para suplir los requisitos de automanejo de la salud de personas trasplantadas fueron las siguientes: monitoreo profesional, adhesión al tratamiento, mantenimiento de las medidas de atención prescritas, búsqueda de orientación e información, repercusiones del diagnóstico, restablecimiento de la inmunidad, inclusión en el mercado laboral, limitaciones físicas y cambios en los hábitos cotidianos. Conclusión: las pautas propuestas reflejan el entendimiento de las necesidades experimentadas para que una persona se adapte al proceso de salud/enfermedad en la fase posterior a un Trasplante de Células Madre Hematopoyéticas e indican la importancia de implementar estrategias que promuevan el automanejo.

RESUMO: Objetivo: conhecer significados e demandas para o autogerenciamento dos cuidados em domicílio de transplantados de Células-Tronco Hematopoéticas e discutir com enfermeiros essas demandas, visando à proposição de diretrizes. Método: estudo qualitativo baseado na pesquisa convergente assistencial desenvolvido entre dezembro de 2020 a agosto de 2021 em um Serviço de Transplante de Células-Tronco Hematopoéticas em Juiz de Fora - Minas Gerais, realizado em duas etapas: entrevista semiestruturada e pequeno grupo de discussão. Participaram 17 pessoas, sendo 12 transplantados e cinco enfermeiros. A análise dos dados foi apoiada pelo software Iramuteq e pela síntese e teorização proposta pela pesquisa convergente assistencial. Resultados: na Etapa 1 realizada com transplantados, emergiram seis classes que foram interpretadas em quatro subcorpus, e apontaram os significados e as demandas para o autogerenciamento. Na Etapa 2, realizada com enfermeiros, as demandas encontradas na Etapa 1 foram refletidas no grupo de discussão e ratificadas. Com base nos achados as diretrizes para atender às demandas de autogerenciamento à saúde de pessoas transplantadas foram: acompanhamento profissional, adesão ao tratamento, manutenção dos cuidados prescritos, busca por orientações e informações, implicações do diagnóstico, restabelecimento da imunidade, inserção no mercado de trabalho, limitações físicas, e mudanças de hábitos cotidianos. Conclusão: as diretrizes propostas refletem a compreensão das necessidades vivenciadas para a adaptação do indivíduo ao processo de saúde/doença na fase pós- Transplante de Células-Tronco Hematopoéticas e expressa à importância da realização de estratégias promotoras ao autogerenciamento.

Educational technologies used to teach self-management after hematopoietic stem cell transplantation: a scoping review / Tecnologías educativas utilizadas para enseñar el autocuidado después del trasplante de células madre hematopoyéticas: revisión de alcance / Tecnologias educacionais utilizadas para o ensino da autogestão no pós-transplante de células-tronco hematopoéticas: scoping review

ABSTRACT Objective: to map the scientific evidence on the educational technologies used to teach self-management in hematopoietic stem cell post-transplantation. Method: a scoping review, based on JBI recommendations. The searches took place between January and February 2022, in databases and repositories of dissertations and theses. The PCC strategy was used, namely: P (Population) - patients (patient participation); C (Concept) - educational technologies and self-management (instructional technology, self-management); and C (Context) - post hematopoietic stem cell transplantation (bone marrow transplantation). Studies that discussed the educational technologies used to teach self-management after hematopoietic stem cell transplantation, available in full electronically, were included. Editorials, letters to the editor and opinion articles were excluded. Duplicate studies were considered only once. The data are presented in figures and chart format. Results: sixteen studies were selected to compose the final sample, most of which showed that the most used educational technologies in the context of hospital discharge after hematopoietic stem cell transplantation are websites, software, movies, online videos or not, care plans, posters, books and booklets aimed at teaching. Conclusion: the use of educational technologies in teaching and patient health education is a reality present in services at any level of health care. The highlight of the approach to this topic is anchored in how these technologies are used and whether they are properly defined for each patient, according to the results of this study.

RESUMEN Objetivo: mapear la evidencia científica sobre las tecnologías educativas utilizadas para enseñar el automanejo en el postrasplante de células madre hematopoyéticas. Método: revisión de alcance, basado en las recomendaciones del JBI. Las búsquedas se realizaron entre enero y febrero de 2022, en bases de datos y repositorios de disertaciones y tesis. Se utilizó la estrategia PCC, a saber: P (Población) - pacientes (participación de los pacientes); C (Concepto) - tecnologías educativas y autogestión (tecnología instruccional, autogestión); y C (Contexto): postrasplante de células madre hematopoyéticas (trasplante de médula ósea). Se incluyeron estudios que discutieron las tecnologías educativas utilizadas para enseñar el autocuidado después del trasplante de células madre hematopoyéticas, disponibles en su totalidad electrónicamente. Se excluyeron editoriales, cartas al editor y artículos de opinión. Los estudios duplicados se consideraron una sola vez. Los datos se presentan en formato de tablas y figuras. Resultados: se seleccionaron 16 estudios para componer la muestra final, la mayoría de los cuales mostró que las tecnologías educativas más utilizadas en el contexto del alta hospitalaria después del trasplante de células madre hematopoyéticas son sitios web, software, películas, videos en línea o no, planes de atención, carteles, libros y folletos destinados a la enseñanza. Conclusión: el uso de tecnologías educativas en la enseñanza y educación en salud del paciente es una realidad presente en los servicios de cualquier nivel de atención a la salud. Lo más destacado del abordaje de este tema está anclado en cómo se utilizan estas tecnologías y si están bien definidas para cada paciente, según los resultados de este estudio.

RESUMO Objetivo: mapear as evidências científicas sobre as tecnologias educacionais utilizadas para o ensino da autogestão no pós-transplante de células-tronco hematopoéticas. Método: scoping review, apoiada nas recomendações do JBI. As buscas ocorreram entre janeiro e fevereiro de 2022, em bases de dados e repositórios de dissertações e teses. Utilizou-se a estratégia PCC, a saber: P (População) - pacientes (participação do paciente); C (Conceito) - tecnologias educacionais e autogestão (tecnologia instrucional, autogerenciamento); e C (Contexto) - pós-transplante de células-tronco (transplante de medula óssea). Foram incluídos estudos que discutissem sobre as tecnologias educacionais utilizadas para o ensino da autogestão no pós-transplante de células-tronco hematopoéticas, disponíveis na íntegra em meio eletrônico. Foram excluídos editoriais, cartas ao editor e artigos de opinião. Os estudos duplicados foram considerados apenas uma vez. Os dados estão apresentados em formato de figuras e quadro. Resultados: foram selecionados 16 estudos para compor a amostra final, dentre os quais, em sua maioria, evidenciaram que as tecnologias educacionais mais utilizadas no contexto de alta hospitalar no pós-transplante de células tronco-hematopoéticas são websites, softwares, filmes, vídeos online ou não, planos de cuidado, cartazes, livros e cartilhas voltados para o ensino. Conclusão: o uso das tecnologias educacionais no ensino e na educação em saúde dos pacientes é uma realidade presente nos serviços em qualquer um dos níveis de atenção à saúde. O ponto de destaque da abordagem a este tema se ancora em como essas tecnologias são utilizadas e se são definidas de forma adequada para cada paciente, conforme resultados deste estudo.