Fatma Burcu Belen

Life-threatening agranulocytosis, anemia, and plasmacytosis after dipyrone use for fever in a child.

Dipyrone or metamizole Na (Novalgin) is commonly used as an antipyretic, analgesic, and spasmolytic agent in some parts of the world; however, it is banned in developed countries because of severe side effects. Here we present a case of a 4-year-old boy who developed life-threatening agranulocytosis, anemia, and marked plasmacytosis in his bone marrow after dipyrone use for fever, which resolved with steroid therapy.

Clinical use of fresh-frozen plasma in neonatal intensive care unit

Recommendations for FFP use in neonates are based on a very limited amount of data, and not on well-designed randomized controlled trials. This retrospective study was performed to analyze our experience with FFP use in neonatal intensive care unit (NICU). From January 2006 until August 2011 a total of 80 neonates were identified as having been treated with FFP. The most common indication for FFP use was prolonged PT or aPTT, representing 32.8% of all usages of FFP. Following FFT treatment PT and aPTT normalized in 42% and 60% patients, respectively. Our results suggest that FFP were often used in acceptable indications in NICU.

Trends in hepatitis B and hepatitis C virus seropositivity among blood donors over 15 years screened in the blood bank of a university hospital

Blood transfusion carries well defined risks including hepatitis B and hepatitis C virus transmission. In this study, records of blood donation candidates between the years 1996-2010 were retrospectively reviewed. A total of 220 841 apparently healthy adult donors were screened for hepatitis B surface antigen, anti-HCV with enzyme linked immunosorbent assay (ELISA) method. The overall prevalence of HbsAg and HCV were 1.07% and 0.39%, respectively. HBV seroprevelance decreased through years 1996-2010 but HCV seroprevelance showed a fluctuant course decreasing from 1996 to 2002. In order to decrease transfusion transmitted infections there should be centralized blood collection systems having qualified staff, equipment and non-remunerated voluntary blood donations must be strongly encouraged.

Molecular basis and bleeding manifestations of factor XI deficiency in 11 Turkish families.

Factor XI (FXI) deficiency is an autosomal bleeding disorder characterized by variable bleeding tendency. In the present study, the gene encoding FXI (F11) was analyzed by direct sequencing in 33 individuals belonging to 11 unrelated Turkish families, and the bleeding tendency was quantitatively assessed by means of a bleeding questionnaire in 27 individuals with low FXI clotting activity and/or mutated F11 gene. We identified 10 distinct mutations (five missense, three nonsense and two splice site), four of which were novel. No mutation was found in one family. Of the four novel mutations, homozygosity for a c.89T>C (p.Phe30Ser) mutation and compound heterozygosity for a c.646G>A (p.Asp216Asn) mutation with the known c.403G>T (p.Glu135*) type II Jewish mutation were associated with severe deficiency, whilst heterozygosity for the novel c.1655A>C (p.His552Arg) and c.1627G>A (p.Glu543Lys) mutations was associated with partial deficiency. p.Glu135* was found in 19% (5/27) of the mutated alleles. Bleeding score was positive in 57% (4/7) of individuals with severe and 39% (7/18) of those with partial deficiency. It was significantly correlated with clinical severity of bleeding (r = 0.43, P = 0.02), but not with FXI clotting activity (P > 0.05). There was no optimal cut-off level of the bleeding score that could predict FXI deficiency. We conclude that the spectrum of mutations found in this study reflects the genetic heterogeneity of FXI deficiency in the Turkish population. Quantitative assessment of the bleeding symptoms by a bleeding questionnaire seems to be useful for evaluating the severity of bleeding episodes, but it can not be recommended as a screening tool for FXI deficiency.

Association of CYP3A5 Expression and Vincristine Neurotoxicity in Pediatric Malignancies in Turkish Population

Vincristine is a widely used chemotherapeutic agent in the treatment of childhood malignancies. Neuropathy is the most common adverse effect. CYP3A4 and CYP3A5 enzymes of cytochrome p450 enzyme system are responsible in vincristine metabolism. Genetic polymorphism may alter the vincristine metabolism and the neurotoxicity rate. In this study, distribution of CYP3A5 alleles among Turkish children with malignancies, relation between CYP3A5 genotype and neurotoxicity rates, as well as severity and duration of neuropathy and total vincristine doses were investigated. Patient group consisted of 115 patients (age, 1 to 17 y) with acute lymphoblastic leukemia and solid tumors, who were treated with vincristine consisting chemotherapy protocols. Control group consisted of 50 children without any neurological symptom or disorders. All patient files were reviewed for presence and severeness of neurotoxicity symptoms. Blood samples were obtained and CYP3A5 genotypes were analyzed. Neurotoxicity occurred in 20.8% of patients. Although it was found to occur more frequently after 4 doses of vincristine, and rates were higher in the low-dose vincristine group suggesting other contributing factors. Although neurotoxicity rate in the CYP3A5*1/*3 genotype was 17.6%, it was 21.6% in the CYP3A5*3/*3 genotype and the difference was not statistically significant (P<0.05). This study suggested that vincristine-related neurotoxicity is dose-independent and genotype is not the only causative factor in the occurrence of neurotoxicity in these patients.

Granulocyte Colony Stimulating Factor Induced Sweet’s Syndrome Following Autologous Transplantation in a Child with Relapsed Acute Myeloblastic Leukemia

Abstract Sweet’s syndrome is characterized by the triad of fever, erythematous skin lesions and neutrophilia. The etiologic factors are quite variable, and granulocyte colony-stimulating factor (G-CSF) use is an extremely rare cause in children with Sweet’s syndrome. We report a G-CSF induced Sweet’s syndrome following autologous transplantation in a child with relapsed acute myeloblastic leukemia.

Platelet usage trends in a tertiary care hospital – Could it be less and less expensive?

Major bleeding is a life threatening complication of severe thrombocytopenia. The aim of this study was to find out the indications and the threshold for platelet transfusions in the pediatric patients of our hospital throughout 1 year. Records of the hospitals blood bank and the files of the patients were retrospectively reviewed. One hundred and four patients, between ages 0-18 years received 378 platelet units. Pretransfusion platelet counts were found to be significantly lower in hematology-oncology groups compared to other clinics (p<0.05). Single donor apheresis was found to be the major source of platelets in hematology (80.8%, n=147) and oncology (86.5%, n=45) clinics. There is a tendency for using apheresis products without proven superiority compared to platelet concentrates in terms of efficacy. This practice can be abandoned by continuous education.

Diagnostic Value of Neopterin during Neutropenic Fever and Determination of Disease Activity in Childhood Leukemias

Neopterin, a pteridine group compound that is secreted from macrophages is shown to be increased in adult leukemia; however there are few studies in childhood leukemia. This study aimed to investigate neopterin levels during childhood leukemia treatment and neutropenic fever episodes for the possibility of using as a marker for disease activity and differentiation of infections. A total of 44 children with acute leukemia, 19 children with infection (control group 1) and 21 healthy children (control group 2) were studied. Median serum neopterin level before induction chemotherapy (day 0) in 25 children (patient group 1) was significantly higher (27.7 nmol/L) than those at the beginning of 30 febrile episodes in 19 children in bone marrow remission (2.2 nmol/L) (patient group 2) and in control group 2 (0.4 nmol/L) (p < 0.05). It was (27.7 nmol/L) also significantly higher in control group 1 than in patient group 2 and control group 2 (p < 0.05). Serum neopterin levels at day 15 (2.1 mmol/L) and day 33 (0.4 mmol/L) of induction were significantly lower than day 0 of ALL subgroup at patient group 1. There were no significant difference in neopterin levels between days 0, 3 and 5 of neutropenic fever as well as between patients with microbiologically and/or clinically documented infections and those with fever of unknown origin in patient group 2 (p > 0.05). Serum neopterin did not show significant correlation with absolute neutrophil count and absolute monocyte count (p > 0.05). In conclusion, elevated neopterin at diagnosis of leukemia with decrement during induction therapy suggest that it might be an indicator of leukemic process; however larger studies for its role in identifying infections are warranted.