Forty patients with high-risk hematologic malignancies, median age 9 years, underwent haploidentical-HSCT from April 2005 to April 2015. Seventeen patients were transplanted with CD3-depleted PBSCs by negative selection (TCD group) following a reduced-intensity conditioning regimen (RIC), and 23 patients received T-cell-replete PBSCs followed by post-transplantation cyclophosphamide (PT-Cy group) after myeloablative conditioning (n=16) or RIC (n=7). Outcomes are reported for the TCD and PT-Cy recipients, respectively. Engraftment was achieved in 88% versus 100%. Median time to neutrophils>500/μL was 10 days versus 15 days. Platelets>20 000/μL occurred at a median of 16 days versus 20 days, respectively. Transplant-related mortality (TRM) was 24% versus 26% at 1 year. The cumulative incidence (CI) of grade III-IV acute GvHD was 7% versus 5%, and chronic GvHD 9% versus 53% (P=0.029). Relapse at 2 years was 31% versus 24%. Actuarial overall survival rates at 2 years were 47% versus 48%. Causes of death were infections (n=3), sinusoidal obstructive syndrome (n=4), acute GvHD (n=2) and relapse (n=9). These results indicate that haploidentical-HSCT is feasible in Uruguay. The TRM rate is of concern and should be the focus of continuing attention. Chronic GvHD risk was higher in the PT-Cy approach, so modifications are justified.

Recently, administration of high-dose intravenous immunoglobulin (HDivIG) either to the mother or the neonate has been proposed in an effort to prevent progressive hemolysis in Rh(D) perinatal disease, but no cases have been published with direct fetal HDivIG administration. We report a case in which HDivIG was repeatedly administered by cordocentesis to a fetus affected by Rh(D) disease from 28 to 36 weeks gestation, at doses of approximately 450 mg/kg. The fetus required no transfusions, and the neonatal evolution was satisfactory. The treatment, performed at three weeks intervals, seemed to be useful in preventing fetal hemolysis. No fetal complications were present. Recurrent administration of HDivIG therapy to the fetus in cases of severe Rh(D) disease, appears to be feasible and free of serious complications to the fetus or the mother.

The York antigen, assigned to the Knops system (KN5-ISBT 022005), is a high frequency antigen present in 90% of the Caucasian and 98% of the African-American population. No cases of anti-Yka in pregnancies have been published. No hemolytic diseases of the fetus and newborn have been observed previously. We report the first case of anti-Yka antibody found in a pregnant woman without fetal anemia, which was monitored by Doppler assessment of peak systolic velocity at the middle cerebral artery. A 36-year-old white woman, gravida 2, para 2 (1994 and 1996) was transfused with two units of packed red cells in 2009. On July 1, 2011 at 13 weeks of gestation of her third pregnancy, “type and screen” showed blood group 0 RhD positive and was found to have one IgG antibody that reacted against all panel red blood cells in the anti-human globulin phase by gel technique. The antibody was identified as anti-Yka and titer was 64. The patient’s phenotype was YK(a–). Peak systolic velocity at the middle cerebral artery, performed by Doppler, at weeks 25, 28, 32 and 34 of gestation did not show fetal anemia. At birth, the newborn was group 0 Rh (D) positive, Yk(a+) with direct Coombs test negative without anemia and hyperbilirubinemia. Our case contributes, as further evidence, to the clinically benignity of the anti-Yka antibody not been a cause of hemolytic disease of the fetus and newborn.

A patient with Sézary syndrome refractory to cytotoxic agents underwent leukapheresis on the Aminco continuous flow centrifuge. Five procedures were performed over a 15-day period. A total of 6.9 X 10(9) cells were removed. The patient's skin lesions and lymphadenopathy regressed. Repeated removal of the buffy coat by leukapheresis has not resulted in thrombocytopenia. It is concluded that cytapheresis may be effective in this clinical condition even when the peripheral leukocyte count is normal.

Hematopoietic Stem Cell Transplantation in Pediatrics: 10 years experienceBackground: In Uruguay the survival estimates for pediatric patients with cancer are comparable to those in developed countries. With the aim of improving outcomes in patients with worse prognosis and provide therapeutic options for treating some non malignant diseases, we started a hematopoietic stem cell transplantation (HSCT) program in 1997, with the fi nancial support of the National Resources Fund (Fondo Nacional de Recursos-FNR). Patients and Methods: The unit was created at the Asociación Española Hospital and counts with three rooms with a sophisticated isolation system. Isolation measures and guidelines for preventing opportunistic infections were strictly enforced. HSCT indications followed the national FNR guidelines according to recommendations of international groups. International standards were used for the patients management. Results: In 10 years 131 transplants were performed in 129 patients. The 10 year overall survival estimate was 43.9 ± 4.6%. Transplant related mortality was 9.3% and the main cause of death was infections. In allogenic HSCT acute graft versus host disease was the leading cause of death. There were three patients who had hither to secondary malignancies. Conclusions: The results, as well as past experience in these 10 years are encouraging. The utility of the procedure in many of the pathologies was analyzed and demonstrated. In the future our goal is to offer this treatment to all patients in which the HSCT is indicated, including those with no compatible family donor. (RESUMEN Introducción: En Uruguay la sobrevida de los niños con cáncer es comparable a la de los países desarrollados. Con el objetivo de mejorar los resultados en pacientes de peor pronóstico y ofrecer opciones terapéuticas curativas a algunas enfermedades no malignas, en el año 1997 se inició un programa de trasplante de progenitores hematopoyéticos (TPH) en pediatría con el fi nanciamiento del Fondo Nacional de Recursos (FNR). Pacientes CONO SUR SOUTH CONE OF AMERICA Rev Chil Pediatr 2010; 81 (3): 275-276Esta sección contiene los artículos originales de las Revistas de Pediatría de las Sociedades de Pediatría del Cono Sur seleccionados en el XIV encuentro de Editores, Brasilia, Brasil 2009, para ser publicados por los países integrantes durante el año 2010.