Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia.

While sickle cell anemia (SCA) and hereditary spherocytosis (HS) share common features of increased spleen erythrophagocytosis due to increased red blood cell (RBC) turnover, SCA is specifically characterized by susceptibility to infections. In this study, histological lesions in the spleens of pediatric patients with SCA were analyzed, in close correlation with past clinical history and comparatively to HS, healthy and transfused ß-thalassemia patients (TDT). An evaluation of red pulp elementary lesions (red pulp fibrosis, iron deposition, number of Gandy-Gamna, and RBC trapping) combined into a severity score was established, as well as B-cell follicles analysis. Quantification on digitalized slides of iron deposition, RBC trapping, and red pulp fibrosis was additionally performed. Spleens from 22 children with SCA, eight with HS, eight with TDT, and three healthy controls (HC) were analyzed. Median age at splenectomy was not different between SCA and HS patients, 6.05 years (range: 4.5-16.0) versus 4.75 (range: 2.2-9.5). Marked heterogeneity was found in SCA spleens in contrast to other conditions. Contrary to previous reports, B-cell follicles were generally preserved in SCA. While RBC trapping was significantly increased in both SCA and HS (compared to TDT and HC), quantitative fibrosis and overall red pulp severity score were significantly increased in SCA spleens compared to other conditions. Moreover, there was an inverse correlation between quantitative fibrosis and number of B-cell follicles, linking these two compartments as well as spleen fibrosis to infectious susceptibility in SCA, potentially through impaired red pulp macrophage scavenging and B-cell subpopulations defects.

Revisión histopatológica de 20 casos de linfoma de Hodgkin infantil (0-14 años) provenientes de áreas de deficiente situación socioeconómica / Histopathological revision of 20 cases of Hodgkin's lymphoma of infants (0-14 years) coming from areas of poor socioeconomical conditions

Los resultados obtenidos en la revisión histopatológica de 20 casos con confirmación de su baja situación socioeconómica han mostrado que la totalidad de los mismos correspondieron a una forma histológica de mejor pronóstico y, por ende, a una aplicación terapéutica de menor agresividad para el paciente, permitiendo a su vez actuar sobre la hipotética causa primaria de la enfermedad con medidas dedicadas a mejorar la respuesta inmune del niño.

The results obtained from histopathological revision of 20 cases with corroboration of their bad socioeconomical condition proof about the total of the casuistry to pertain into a good prognostic histological type, and for this reason submitted to a low aggresive chemotherapy treatment for the patient, and consecutively to put in action the therapeutic to restore the immunological response.