Haploidentical hematopoietic stem cell transplantation with αβTCR+/CD19+ depletion in pediatric patients with malignant and non-malignant disorders.

Haploidntical hematopoietic stem cell transplantation has been increasingly used in recent years for patients without a matched donor. The αβTCR+/CD19+ depletion technique provide a graft that is enriched with CD34 cells, γδ-T-cells and natural killer. The current experience with αβTCR+/CD19+ depleted grafts in pediatric patients with malignant and non-malignant disorders, demonstrated rapid engraftment, improved immune reconstitution and low risk of GVHD.

Nephrectomy in children with wilms' tumor: 15 years of experience with "Tumor Delivery Technique".

Background: The contemporary surgical approach to Wilms' tumors follows that used in adults with renal cell carcinomas, namely, early occlusion of the renal vessels and then removal of the kidney as an intact mass. For years, the surgical approach at our institution has been different, starting with blunt separation of the kidney from the surrounding tissues, followed by its delivery outside the abdominal cavity while it is only attached to the major blood vessels which are subsequently ligated. We aimed to present this "tumor delivery technique" and evaluate its outcomes.

Comparison of two cytoreductive regimens for αβ-T-cell-depleted haploidentical HSCT in pediatric malignancies: Improved engraftment and outcome with TBI-based regimen.

Background: Graft manipulation using selective depletion of αβ-T cells provides a source of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) enriched in effector cells. We report our experience implementing this haplo-HSCT for high-risk malignancies in pediatric patients focusing on the conditioning regimen.

Procedure: We performed a retrospective study of patients who underwent T-cell receptor αβ-depleted haplo-HSCT for high-risk pediatric malignancies.

Spirometry follow-up in young children with hemato-oncologic diseases.

Background: Treatment of hemato-oncologic patients is often associated with severe pulmonary complications. Pulmonary function is routinely evaluated in older children, whereas in young patients, forced spirometry measurements are infrequently performed.

Aim: To assess severity of airway disease using forced spirometry measured prior to and after treatment (over 3 years) in hemato-oncologic children aged 3-7 years in comparison to a healthy population.

Pulmonary function studies in children treated by chemoradiotherapy and stem cell transplantation.

Background: Management of pediatric patients with malignant and hematological diseases is frequently associated with pulmonary complications. We assessed pulmonary function at diagnosis and during a 5-year follow-up to identify risk factors associated with pulmonary deterioration.

Procedure: Ninety patients (age range 3-20) who were treated at the Pediatric Hematology-Oncology Department, Sheba Medical Center, Israel, were entered into the study.

Congenital amegakaryocytic thrombocytopenia-3 novel c-MPL mutations and their phenotypic correlations.

Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare bone marrow failure syndrome associated with thrombocytopenia and a tendency to progress to aplastic anemia. Mutations in the c-MPL gene encoding for thrombopoietin receptor have been identified in the majority of the patients. Previous studies suggest a genotype-phenotype correlation wherein the severity of the disease depends on the type of mutation present and residual thrombopoietin receptor activity.