Stem cell transplantation for congenital dyserythropoietic anemia : an analysis from the European Society for Blood and Marrow Transplantation

Non peer reviewe

The experience of using defibrotide for the prevention and treatment of hepatic veno-occlusive disease after pediatric hematopoietic stem cell transplantation: A single center experience

44th Annual Meeting of the European-Society-for-Blood-and-Marrow-Transplantation (EBMT) -- MAR 18-21, 2018 -- Lisbon, PORTUGALWOS: 000487702807051…European Soc Blood & Marrow Transplanta

The experience using of eltrombopag for treatment of thrombocytopenia after allogeneic hematopoetic stem cell transplantation in pediatric patients

44th Annual Meeting of the European-Society-for-Blood-and-Marrow-Transplantation (EBMT) -- MAR 18-21, 2018 -- Lisbon, PORTUGALWOS: 000487702807052…European Soc Blood & Marrow Transplanta

The experience of BK polyoma virus infection treatment after allogeneic hematopoietic stem cell transplantation in pediatric patients

44th Annual Meeting of the European-Society-for-Blood-and-Marrow-Transplantation (EBMT) -- MAR 18-21, 2018 -- Lisbon, PORTUGALWOS: 000487702807050…European Soc Blood & Marrow Transplanta

Treatment and risk factors of hepatic veno-occlusive disease after pediatric hematopoietic stem cell transplantation: A single-center experience

45th Annual Meeting of the European-Society-for-Blood-and-Marrow-Transplantation (EBMT) -- MAR 24-27, 2019 -- Frankfurt, GERMANYWOS: 000487707800323…European Soc Blood & Marrow Transplanta

The Prognostic Significance of Serum Tumor Necrosis Factor (TNF)-Related Apoptosis-Inducing Ligand (TRAIL) in Childhood Acute Leukemias

WOS: 000363969000016Purpose: Tumor necrosis factor (TNF)-related apoptosis-inducing ligand (TRAIL) is a TNF superfamily member. TRAIL is transmembrane protein expressed on cell surfaces and has a broad expression pattern in a variety of organs. Cleavage of its C-terminal part (extracellular domain) allows for a soluble form of TRAIL. TRAIL induces apoptosis with its receptors TRAIL-receptor 1 (TRAIL-R1), TRAIL-receptor 2 (TRAIL-R2) however apoptosis can not be induced by receptors TRAIL-receptor 3 (TRAIL-R3) and TRAIL-receptor 4 (TRAIL-R4). There are many trials to search the correlation between leukemia and apoptotic pathway disorders. In this study we determined the seum levels of TRAIL in acute childhood leukemias at first diagnose. We aimed to determine the relation between the levels of serum TRAIL and patient's survey, clinical parameters. Material and Methods: The study was performed in patients admitted to Pediatric Hematology and Pediatric Oncology Department of Cukurova University Medical Faculty between October 2009 and July 2010. Twenty-three cases with new diagnosis acute lymphoblastic leukemia (ALL) at the age disturbition 9-months-12-year and 8-months and fourteen cases with new diagnosis acute myeloblastic leukemia (AML) at the age disturbition of 9 days-18 years are included in this study. Twenty-one healty children with no blood disease with similar sex and age with leukemia group was chosen as the control group. Serum TRAIL levels were determined by using ELISA method. Results: The comparison of the average values of the TRAIL levels in acute leukemia patients and control group have shown that patients with leukemia have low serum TRAIL levels (p = 0.002). In patients with high-risk-grade (HRG) of ALL compared with control group have shown low serum TRAIL levels in HRG of ALL (p = 0.008). In patients with common acute lymphoblastic leukemia antigen(CALLA)(-) B ALL compared with control group have shown low serum TRAIL levels in CALLA(-) B ALL (p = 0.004). Children with acute leukemias (ALL, AML) who died during treatment compared with survived group have shown low levels of serum TRAIL in expired patients (p = 0.004). Conclusion: As a result, serum TRAIL might play a role in leukomegenesis. The low levels of serum TRAIL detected in our patients may be associated with leukomogenezis and impaired TRAIL-mediated apoptosis. To suggest soluble TRAIL's role in acute leukemias detection of TRAIL-mediated apoptosis is needed. The low serum TRAIL may be used as a sign of bad prognosis. For more comphrensive results prospective studies with greaater number of patients are needed

Correlation between HJHS and HEAD-US Scores during Prophylaxis in Turkish Patients with Hemophilia: National Prospective Observational Study

WOS: 000536674800086[No abstract available

Bilateral recurrent external obturator muscle hematoma: An unusual cause of pelvic pain in hemophilia

WOS: 000453162300036Following joint hemorrhages, intramuscular hemorrhages are the second most prevalent bleeding pattern in hemophiliac patients. Hematomas of the iliopsoas muscle are a well-known complication of hemophilia; however, obturator muscle hematomas are rare. We herein report a case of spontaneous bleeding of the bilateral external obturator muscles, which occured three times within a period of 9 months in a hemophilia patient with factor VIII inhibitors. To the best of our knowledge, this is the first published case of an obturator externus muscle hematoma in hemophilia. In addition to hip hemarthrosis, iliopsoas hematomas and acute appendicitis, obturator muscle hematoma should be considered as one of the diagnostic alternatives for pelvic pain in hemophilia. patients. Magnetic resonance imaging enables rapid diagnosis of obturator muscle hematoma

Visceral Leishmaniasis in Children in Southern Turkey: Evaluation of Clinical and Laboratory Findings and Liposomal Amphotericin B Treatment

WOS: 000490575700006Aim: Visceral leishmaniasis (VL) is a systemic infection that spreads hematogenously and affects the reticuloendothelial system by the infection of macrophages. VL occurs commonly in children, and only rarely in adults. VL should be considered in patients with prolonged high fever, hepatosplenomegaly, pancytopenia, weight loss, pallor and hypergammaglobulinemia. Materials and Methods: In this study, a total of 18 pediatric patients -9 (50%) males and 9 (50%) females- treated for VL at our clinic from January 2004 to July 2014 were analyzed retrospectively. Average time from symptom onset to hospital admission was 64 +/- 21 days (range: 30-100 days). The mean age of patients was 88 +/- 40 months (range: 36-182 months). Results: The most common symptom at presentation was fever (88.9%). Other common symptoms were fatigue, chills, weight loss and anorexia. Physical examination revealed splenomegaly and hepatomegaly in all patients. Anemia (92.4%), leukopenia (78.7%) and thrombocytopenia (76.2%) were the most prominent laboratory abnormalities and 82.2% of the patients were pancytopenic on admission. Bone marrow smear was positive for leishmania in 100% of the patients. All patients received treatment with liposomal amphotericin B. Conclusion: In certain regions, increased humidity rates associated with construction of dams and irrigation canals may lead to changes in the ecological balance and thus cause an increase in the population of disease-spreading vectors. Additionally, recent migration from the middle-eastern region to western parts of the world due to regional civil wars may have contributed to the observed increase in the incidence of various diseases such as VL