Vol 16, No 1 (2017)

In this study we evaluated incidence and risk factors of Herpesvirus infections – Cytomegalovirus (CMV) и Epstein–Barr (EBV) – in recipients of allogenic hematopoietic stem cell transplantation (alloHSCT) with TCRαβ and CD19 depletion of graft. The usage of this method results to graft preservation of non-alloreactive TCRgd lymphocytes, leading to decresed risks of graft versus host disease (GVHD) with adequate antiviral control. We estimated the outcomes of 182 HSCT from haploidentical and unrelated donors in patients younger than 23 years old with malignant anf nonmalignant disoders. The cumulative incidence (Cum Inc) of CMV infection was 51% (95% CI 44–60). By uni- and multivariate analyzes was found the significant influence on CMV-viremia of acute GVHD grade 2–4, recipient’s seropositivity before HSCT and patient’s malignant disoder. Cum Inc of EBV infection was 33% (95% CI 26–42). The risk factor of EBV viremia was acute GVHD grade 2–4. The incidence of CMV-disease was 6% (11 patients), EBV-related posttransplant lymphoproliferative disoder (PTLD) 0,5% (1 patient). CMV and EBV viremia did not decrease survival after HSCT. 
TCRαβ and CD19 depletion of graft was associated with significant risk of CMV viremia in patients after alloHSCT, but did not influence survival and transplant related mortality, and with extremely low risk of PTLD development.

Myeloablative chemotherapy with autologous hematopoietic stem cells transplantation, can improve the results of long-term survival in patients with high-risk neuroblastoma. This treatment requires a sufficient number of autologous peripheral blood stem cells. Methods: 118 apheresis procedures was completed for 116 children with high-risk neuroblastoma, the median age was 2 years and 10 months (range, 6 months to 13 years), median body weight of 13 kg (range, 5.8 to 47 kg). Mobilization with G-CSF at a dose of 10 mg/kg was initiated at the time of incipient recovery of hematopoiesis after the last cycle of chemotherapy, monitoring of CD34+ cells in peripheral blood were started on 3rd day from the beginning of mobilization. Apheresis was performed the following day after reaching the number of CD34+ cells >15 cells/μl after 4 consequent days of mobilization. Results: Modification of mobilization, based on CD34+ cells monitoring data, allowed us to reach the median number of CD34+ cells of 105,27 cells/μl (range, 14.8–714.8) at the day of apheresis. The median number of collected CD34+/kg was 14,43×106/kg b.w. (range, 2.68–74). Was revealed a significant correlation between the dose of CD34+/kg b.w in the apheresis product and the level of CD34+ cells in peripheral blood at the day of apheresis (R=0.762; р<0.0001) and decrease of the results of mobilizing results and apheresis efficiency if mobilization was initiated later than after the 3rd course of chemotherapy. Conclusion: By monitoring the number of circulating CD34+ cells in peripheral blood and modification of mobilization tactic, the task to collect an adequate dose of CD34+ cells for autoSCT with one apheresis procedure was feasible. In 88 (75.86%) of the 116 patients we were able to collect 10×106/kg CD34+ cells and more, and 100% of patients collected > 2.5×106/kg CD34+ cells with 1 apheresis.

Trichosporon fungus infection causes systemic disease in immunocompromised patients, including those in the departments of hematological profile. This article provides an overview of the literature and case report of this infection in a child with acute lymphoblastic leukemia.

Differential diagnosis of adrenal gland tumor in neonates and children of the first months of life is an extremely difficult clinical task. Most often, it is congenital cystic neuroblastoma or prenatal adrenal hemorrhage. As a rule, it is hard to make the right diagnosis due to absence of clinical presentation and similarity of visualization data therefore sometimes surgical tumor removal followed by histological verification becomes the method of choice. The article presents a clinical case of the adrenal gland tumor in 5-months old boy. Authors discuss the difficulties in the formulation of the final clinical diagnosis.

Inherited functional platelet disorders (IFPD) are very heterogeneous group of hemorrhagic diseases that are difficult to diagnose. Prevalence of IFPD in general population is probably underestimated. In most cases IFPD cause mild to moderate bleeding symptoms in everyday life but may result in life-threating bleeding after trauma and surgical interventions. Rational diagnostic approach is critical to prevent these complications. Data on diagnostic algorithms as well as on benefits and disadvantages of specific laboratory methods are presented in this review. Approach to clinical evaluation is also reviewed with particular attention to bleeding assessment tools. The main emphasis is made on light transmission aggregometry, measurement of platelet granules content and release, flow cytometry. Problems of standardization of platelet functional tests are outlined. Diagnostic algorithms are applied according to the up to date recommendations of International Society on Thrombosis and Haemostasis.