No abstract

Inherited bleeding disorders (IBDs) are caused by quantitative and qualitative alterations of either platelets or plasma proteins involved in coagulation and fibrinolysis. Hemophilias are the most frequent IBDs; however, accumulated data from various studies reported that von Willebrand disease (VWD) is the most common cause of IBD, with an increased incidence of platelet function defects, mostly due to the increased rate of consanguinity in some communities. VWD is an inherited disorder of homeostasis due to quantitative or qualitative defect of von Willebrand factor. Data on its epidemiology and impact in developing countries are limited. The objective of this study was to assess the local prevalence of some IBD and establish the clinical and historical variables that are predictive for those bleeding disorders in pediatrics. The study involved 43 children with various bleeding manifestations and 15 age- and sex-matched controls, recruited from the Pediatrics Hematology Clinic at the National Research Centre, Sausan Mubarek children's hospital in Cairo, Egypt and the King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Hematological profile included platelet counts and function, prothrombin time, partial thromboplastin time, factor VIII antigen and its activity, factor IX antigen and its activity, von Willebrand factor and its activity assayed with multimeric analysis. A total of 12 (27.9%) children had VWD, 11 (25.5%) had hemophilia A, three (7%) had hemophilia B, seven (16.3%) had platelet dysfunction and 10 (23.3%) had bleeding with undiagnosed cause. Two of the VWD cases had type I, three had type II, four had type III and one case appeared to have type IIM and another to have IIB VWD. Bruising and epistaxis were the main symptoms in all children with VWD The majority of platelet dysfunction disorders were diagnosed as Glanzmann's thrombasthenia. VWD and Glanzmann's thrombasthenia should be considered not uncommon causes of IBDs in children in Egypt and Kingdom of Saudi Arabia. Routine hematological screening should be mandatory in children with positive family history of bruising and bleeding as a predictor for IBD.

We report a retrospective analysis of Cytomegalovirus (CMV) infection: incidence, recurrence, resistance, and subsequent disease of 81 children who underwent allogenic hematopoietic stem cell transplantation (HSCT). The recipient and/or donor's CMV serology was positive prior to transplant [recipient (R+) and/or donor (D+)]. CMV was monitored by RT-PCR starting from the first week post transplant. Forty patients showed CMV infection (49, 5%). Of them 10 manifested CMV disease leading to four deaths. In univariate analysis, factors associated with CMV infection were CMV R+ P < .01, CMV R+/D+ pair P < .01, nonbone marrow (BM) stem cell source P < .05, nonirradiation conditioning regimen P < .05, Antithymocyte globulin (ATG) P < .01. Factors associated with CMV resistance were: >1 HLA allele mismatch P < .05, CMV R +/D-pair P < .01, CMV D-P < .01, non-BM P < .05, nongenoidentical transplant P < .01. CMV disease was influenced by >1 HLA allele mismatch (P < .001), non-BM (P < .01). On multivariate analysis, CMV R+/D- (P < .05), corticosteroids ≥2 mg/kg P < .01, ATG P < .01 and non-BM (P < .05) were independent factors for CMV infection. CMV R+ transplant is associated with more CMV infection and resistance to preemptive treatment. Prolonged immune suppression (IS) worsens outcome of CMV infection and should be shortened whenever possible.

We aimed for the comparison of two protocols (OAP and COMP) as chemotherapy treatment in children with Hodgkin lymphoma (HL). A total of 119 children newly diagnosed with HD were divided to receive either the anthracycline-based OAP protocol or the alkylating-agent-based COMP protocol. Sixty patients received the OAP protocol and 59 patients received the COMP protocol. Complete response was achieved for 81.4% of patients treated with the COMP protocol versus 53.3% for those who received the OAP treatment. Toxic hepatitis or liver cell failure was recorded in 5% of patients treated with the COMP protocol. Complications were more frequent in those treated with the OAP protocol, as 6.8% developed heart failure and 20% showed toxic hepatitis or liver cell failure. The relapse rate was almost equal in both treatment arms. Patients treated with the COMP protocol achieved a better response and less toxicity but with similar survival to those given the OAP protocol.

No abstract