From January 1991 to June 2009, 245 children with beta thalassemia major who underwent their first allogeneic HSCT in Turkey and who were followed for a minimum of one yr post-transplantation were enrolled this study. The median age of the patients was 6.6 yr old (range, 1-22 yr). The distribution of Pesaro risk class I, II, and III categories was 41, 130, and 63 children, respectively. The median serum ferritin level was 2203 ng/mL. Eighty-eight patients received bone marrow (BM) stem cells; 137, peripheral blood (PB) stem cells; and 20, cord blood (CB) stem cells. The donors were HLA-matched siblings or parents. Median engraftment times were shorter in PBSCT patients compared with the BMT group (p < 0.001). Grade II-IV acute GvHD was observed in 33 children (13.5%), while cGvHD was observed in 28 patients (12.5%), eight of whom had the extensive form. Thalassemic reconstitution was observed in 43 (17%) of the transplant patients. Post-transplant aplasia occurred in three patients, and the TRM rate was 7.75%. Seventeen patients were lost after 100 days. The thalassemia-free survival and OS rates were 68% (95% CI, 61.8-74.2) and 85.0% (95% CI, 80.2-89.8), respectively. We believe that this study is important because it is the first multicenter national data for children with beta thalassemia major receiving HSCT.

This study analyzes the data of thrombotic children who were followed up in different pediatric referral centers of Turkey, to obtain more general data on the diagnosis, risk factors, management, and outcome of thrombosis in Turkish children. A simple two-page questionnaire was distributed among contact people from each center to standardize data collection. Thirteen pediatric referral centers responded to the invitation and the total number of cases was 271. All children were diagnosed with thromboembolic disease between January 1995 and October 2001. Median age at time of first thrombotic event was 7.0 years. Of the children 4% of the cases were neonates, 12% were infants less than 1 year old, and 17% were adolescents. Thromboembolic event was mostly located in the cerebral vascular system (32%), deep venous system of the limbs, femoral and iliac veins (24%), portal veins (10%), and intracardiac region (9%). Acquired risk factors were present in 86% of the children. Infection was the most common underlying risk factor. Inherited risk factors were present in 30% of the children. FVL was the most common inherited risk factor. Acquired and inherited risk factors were present simultaneously in 19% of the patients. Eleven children had a history of familial thrombosis. Due to the local treatment preferences, the treatment of the children varied greatly. Outcome of the 142 patients (52%) was reported: 88 (62%) patients had complete resolution, 47 (33%) had complications, 12 (9%) had recurrent thrombosis, and 34 (24%) died. Three children (2.1%) died as a direct consequence of their thromboembolic disease. The significant morbidity and mortality found in this study supports the need for multicentric prospective clinical trials to obtain more generalizable data on management and outcome of thrombosis in Turkish children.

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The aim of this study was to investigate the changes in the peripheral blood of newborns of hypertensive mothers. The umbilical cord blood from newborns of 31 hypertensive mothers and 32 healthy mothers were examined. In all subjects, complete blood count, peripheral blood smear, reticulocyte count, vitamin B12, folate, ferritin levels and hemoglobin electrophoresis were performed. The subjects were followed up on for 1 year in terms of infections. RBC, hemoglobin, reticulocyte count and normoblast count were higher in the newborns of hypertensive mothers compared to the control group, and total leukocytes, neutrophil, lymphocyte, monocyte, eosinophil, and thrombocyte counts were lower. The number of neutropenic and thrombocytopenic subjects in newborns of hypertensive mothers was higher compared to the control group. On peripheral smears, dysplastic changes in neutrophils and erythrocytes were observed with a higher rate in newborns of hypertensive mothers compared to the control group. HbF levels were found to be higher in newborns of hypertensive mothers compared to the control group. During the follow-up period of 1 year, the number of infections in newborns of hypertensive mothers was found to be higher than the control group. Conclusion: Newborns of hypertensive mothers should be carefully evaluated and monitored in terms of hematologic abnormalities. Complete blood counts and peripheral blood smears can be used as significant parameters for early diagnosis of possible complications.

Linear IgA disease is characterized by the presence of linear IgA deposits in the basement membrane zone of the skin, and circulating basement membrane zone antibodies are detected in 80% of cases. The disease occurs in both adults and children, and is designated adult linear IgA disease in the former and chronic bullous disease of childhood (CBDC) in the latter. We describe a 5-year-old boy with acute lymphoblastic leukemia in remission, in whom CBDC developed after treatment with trimethoprim/sulfamethoxazole (cotrimoxazole). To our knowledge, this is the first reported case of possible drug-induced CBDC.