Haploidentical Stem Cell Transplant with Post-Transplant Cyclophosphamide in Pediatric Hemophagocytic Lymphohistiocytosis.

PURPOSE: Primary hemophagocytic lymphohistiocytosis is a severe and uncommon disease affecting pediatric patients. Genetic abnormalities have been related to altered apoptosis and exaggerated inflammatory reactions. Chemoimmunotherapy and stem cell transplantation are treatment options, but transplant is the only curative treatment. Here we aim to describe the treatment with hematopoietic stem cell transplantation with a novel strategy and the outcomes. METHODS: An observational, descriptive, case series study was performed in pediatric patients of two high complexity medical centers in Colombia. Data was collected retrospectively between 2015 and 2020. RESULTS: We describe five pediatric cases with a diagnosis of primary hemophagocytic lymphohistiocytosis. All were treated with replete-cell haploidentical hematopoietic stem transplantation, reduced-intensity conditioning, and post-transplant cyclophosphamide, in two high-complexity centers in Colombia. All patients are alive, and one is receiving management for chronic graft-versus-host disease. CONCLUSION: To the best of our knowledge, there are few reports in the literature with this strategy, promising a possible alternative when there are no other donor options.

Meta-analysis of hematopoietic stem cell transplantation in major histocompatibility complex class II deficiency.

Major histocompatibility complex class II deficiency is a rare case of PID. Specific recommendations for hematopoietic stem cell transplant, the only curative treatment option, are still lacking. This meta-analysis aims to identify the factors associated with better prognosis in these patients. Thirteen articles reporting 63 patients with major histocompatibility complex class II deficiency that underwent hematopoietic stem cell transplant were included. The median age for hematopoietic stem cell transplant was 18 months. The most common source of transplant was bone marrow, with alternative sources as umbilical cord blood emerging during recent years. The highest proportion of engraftment was seen with umbilical cord. Engraftment was higher in patients with matched donors, with better overall survival in patients with reduced-intensity conditioning. Graft-vs-host disease developed in 65% of the patients, with grades I-II being the most frequently encountered. There was a higher mortality in patients with myeloablative conditioning and no engraftment. There was an inverse correlation between survival and stage of graft-vs-host disease. The main cause of mortality was infectious disease, mostly secondary to viral infections. Ideally, matched grafts should be used, and reduced-intensity conditioning should be considered to reduce early post-transplant complications. GVHD and viral prophylaxis are fundamental.

Hemophagocytic Lymphohistiocytosis Associated with Salmonella typhi Infection in a Child: A Case Report with Review of Literature.

We present the case of an 8-year-old girl with hemophagocytic lymphohistiocytosis secondary to a Salmonella typhi infection. She received antibiotic treatment and intravenous immunoglobulin with complete resolution of the symptoms. We present a review of previously reported pediatric cases and propose a gradual approach to treatment.

Morbimortalidad en la unidad de cuidados intensivos pediátricos del Hospital Universitario San Vicente de Paúl, Medellín, Colombia, 2001-2005 / Morbidity and mortality at a pediatric intensive care unit in Medellín, Colombia, 2001-2005

Introducción: el aumento en el número y la complejidad de las Unidades de Cuidados Intensivos Pediátricos (UCIP), sumado a su alto costo, ha estimulado el interés y la necesidad de evaluar su funcionamiento, la eficacia de la labor realizada y la calidad de los servicios que prestan, mediante indicadores asistenciales que se derivan de estudios descriptivos de morbimortalidad. Objetivo: describir las causas de morbilidad y mortalidad y las características sociodemográficas de los niños admitidos a la UCIP del Hospital Universitario San Vicente de Paúl (HUSVP) de Medellín, Colombia, en el período comprendido entre el primero de enero de 2001 y el 31 de diciembre del 2005. Metodología: estudiodescriptivo y retrospectivo de corte transversal, en el que por medio de una aleatorización simple se hizo la selección de 328 historias de pacientes que ingresaron a la UCIP duranteel período de estudio. Se tabularon y analizaron estadísticamente los datos obtenidos de la revisión de las historias. Resultados: se encontró que 59,8% de los pacientes eran de sexo masculino, 40,9% eran menores de 1 año y 43% provenían del área metropolitana del valle de Aburrá. La edad promedio fue 44 meses. Solamente 47% tenían afiliación a la seguridad social. Las principales causas de admisión a la UCIP fueron: infección respiratoria(23,8%), ingresos postquirúrgicos (23,5%), falla respiratoria (18,6%), choque séptico (16,5%) y malformaciones congénitas (13,1%); el promedio de estancia fue 5 días. Requirieron ventilación mecánica 52,4% de los pacientes, 26% de los cuales presentaron alguna complicaciónderivada de ella. La mortalidad general fue 21,6% y los pacientes de sexo masculino miprovenientes del área metropolitana fueron los más afectados. Las principales causas de muerte fueron:falla orgánica múltiple (26,8%) y choque séptico (19,7%). 56% de las muertes ocurrieron durante las primeras 48 horas después de la admisión a la UCIP...

Introduction: increase in the number andcomplexity of pediatric intensive care units (PICU), added to their high cost, have stimulated interest in evaluating their functioning, efficiency and qualityby means of markers derived from descriptive studies of morbidity and mortality. Objective: to describe the causes of morbidity and mortality, as well as the social and demographic characteristics of patients admitted to the PICU of Hospital UniversitarioSan Vicente de Paúl (HUSVP) in Medellín, Colombia, between January 1, 2001 and December 31, 2005. Methodology: descriptive and retrospective cross section study. 328 patient charts were randomly selected and then reviewed. All information wastabulated and statistically analyzed. Results: 59.8% of the patients were males, 40.9% were under oneyear of age, and 43% came from the metropolitan area around Medellín. Average age at admission was 44 months. Only 47% of the patients had some type of health insurance. The main causes for admission to the PICU were: respiratory infection (23.8%), postsurgical cases (23.5%), respiratory failure (18.6%), septic shock (16.5%) and congenital malformations (13.1%). Average length of stay was 5 days.Mechanical ventilation was required by 52.4%, and 26% of them suffered from some complication. Mortality was 21.6%, affecting mainly male patientsfrom the metropolitan zone around Medellín. 56% of the deaths occurred during the first 48 hours after admission to the PICU. The main causes of deathwere multiple organ failure (26.8%) and septic shock (19.7%). Discussion: infants are the group at greater risk for admission to a PICU, most frequently due to respiratory and infectious problems, which may reflect faults in the promotion and prevention programs at the lowest levels of the health caresystem. Mortality was high in comparison with that at other PICUs in Latin America...