Akut Lenfoblastik Lösemide İmmunfenotipleme ve Klinik ile İlişkisi

ÖZ Amaç: Akut lenfoblastik lösemi (ALL) tanılı çocuk hastalarda immunfenotipleme ve klinik özelliklerle ilişkisini değerlendirmek. Yöntem: Merkezimizde 1989-2007 yılları arasında ALL tanısı almış ve tedavi edilmiş olan 18 yaş altındaki 192 hastada akım sitometri ile incelenen immunfenotipleme sonuçlarının klinik özellikler ile ilişkisi değerlendirildi. Bulgular: Vakaların 79’unu (%41,1) kız, 113’ünü (%58,9) ise erkek hastalar olusturmaktaydı. Vakalar prekürsör-B hücreli ve T hücreli ALL olarak 2 gruba ayrıldı. T hücreli ALL erkeklerin %27.6’sında görülürken, bu oran kızlarda %14,2 idi (p=0,038). Yaş grupları immunfenotiplendirmeye göre değerlendirildiğinde, 1 yaş altında tüm hastalar prekürsör-B ALL grubunda iken, 1-10 yaş arasındaki vakaların %82,9’u prekürsör B ALL, %17,1’i T-ALL’li grubunda, ≥10 yaş üzerindeki vakaların %58,6’sı prekürsör B ALL’li, %41,4’ü T-ALL grubunda idi (p: 0.002). Hiperlökositoz, ekstramedüller tutulum, lenfadenopati ve mediastinel kitle T hücreli ALL grubunda anlamlı olarak daha sık görüldü. T hücreli ALL sıklığının yıllar içinde azaldığı görüldü. Sonuç: Akut lenfoblastik lösemi erkeklerde kız cinsiyete göre daha fazla görülmektedir. Erkek çocuklarda ve 10 yaş üstünde T hücreli ALL sıklığı artmaktadır. Lenfadenomegali, mediastinel kitle, ekstramedüller tutulum ve hiperlökositoz sıklığı, T hücreli ALL grubunda prekürsör B hücreli ALL grubuna göre anlamlı olarak artmaktadır. T hücreli ALL sıklığının yıllar içinde azaldığı bunun sosyoekonomik düzeyde iyileşme ile ilişkili olabileceği düşünülmüştür

First-Step Results of Children Presenting with Bleeding Symptoms or Abnormal Coagulation Tests in an Outpatient Clinic

Objective: Mild bleeding symptoms are commonly seen in the general population. The aim of this study was to determine the final clinical and laboratory features of children referred for a first evaluation with a suspected bleeding disorder in the pediatric outpatient clinic of İstanbul University. Materials and Methods: The medical records of 26,737 outpatients who were admitted to the Division of Ambulatory Pediatrics between 31 October 2011 and 31 October 2012 were evaluated retrospectively. Ninety-nine patients were initially diagnosed as having probable bleeding disorders and were followed up. The symptoms of bleeding in addition to coagulation tests were analyzed. Results: Of the 99 patients, 52 (52.5%) were male and 47 were female, and the mean age of the entire study group was 9.1±4.1 years (minimum-maximum: 2-18 years). Major bleeding symptoms were epistaxis in 36 patients (36.4%), easy bruising in 32 (32.3%), and menorrhagia in 6 (6.1%). After initial tests ordered by the pediatrician, 36 of 99 patients (36.4%) were diagnosed as having bleeding disorders that included von Willebrand disease in 12 (12.1%), hemophilia A or B in 9 (9.1%), and other rare factor deficiencies in 9 (9.1%). Six patients (6.1%) were found to have combined deficiencies. Seven of 36 patients had a family history of bleeding. Conclusion: Among the patients referred for bleeding disorders, 36.4% were diagnosed with a bleeding disorder with the help of primary screening tests ordered in the outpatient clinic

Risk factors and clinical characteristics of virus Infection after haematopoietic stem cell transplantation

Risk factors and clinical characteristics of virus Infection after haematopoietic stem cell transplantation Gamze Kalın Ünüvar*1, Zeynep Ture Yuce1, Aysegül Ulu Kilic2 1University Of Erciyes Faculty Of Medicine, Kayseri, Turkey, 1University Of Erciyes Faculty Of Medicine, Kayseri, Turkey Background: BK polyomavirus is an important cause of morbidity and mortality in hematological patients after hematopoietic stem cell transplantation (HSCT). It is acquired in childhood and especially becomes latent in urothelial epithelial cells. Reactivation of virus after HSCT can be seen with asymptomatic viruria or hemorrhagic cystitis (HC). The aim of the study was to assess risk factors, clinical characteristics and treatment options of BK virus infection after HSCT. Materials/methods: We retrospectively analyzed information about patients with HSCT and BK virus (BKV) disease between January 2017-August 2019. Data included; underlying hematological disease, transplantation type, associated graft versus host disease (GVHD) and recent use of immunosuppressive agent. Results: In total fifty-eight patients with HSCT were evaluated and BKV disease occurred in 20 (34%). The median age was 40 (range, 20 to 68), 50% were male. The most underlying disease was Acute Myeloid Leukemia (n=11). Five patients had autologous and fifteen patients had allogeneic SCT. The median time to engraftment was 15 days (range, 10 to 20). GVHD was seen eleven patients (40% skin, 15% gastrointestinal GVHD). These patients received systemic glucocorticoid therapy or immunosuppressant agents. The median time elapsed to BK virus disease after HSCT was 60 days (range, 30 to 450). Sixteen patients with BKV disease had high grade (grade 3) HC and four patients had low-grade HC (grade 2). While BK viremia was positive in 17 patients (68%), viruria was positive for all patients. Eight patients (15%) were treated with ciprofloxacin and cidofovir combination, six patients (30%) with cidofovir and three patients (15%) with ciprofloxacin. Three of them (20 patients) was treated by intravesical cidofovir. The complete response to the viruria or viremia was obtained from 11 patients (55%). Conclusions: HC associated with BKV is an emerging clinical problem after HSCT causing prolonged hospitalization and mortality. It can be severe because the treatment options are often ineffective. The main goal of treatment is to reduce the dose of immunosuppressive agents. Close monitoring of BK virus in high-risk patients can be an important method to improve the complication in the early period.</p