Paroxysmal nocturnal hemoglobinuria in the differential diagnosis of thrombocytopenia

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease which diagnosis may be delayed due to variable clinical findings. We describe herein a case of PNH in a 21 year old woman who admitted with complaints of chronic weakness, intermittent spontaneous ecchymoses, and an intermittent abdominal pain. On laboratory tests thrombocytopenia and iron deficiency anemia without any clinical findings were found. Flow cytometric evaluations showed a PNH clone of 15% for erythrocytes, 64% for monocytes, and 60% for granulocytes. The patient was diagnosed with PNH and an eculizumab therapy was initiated. Following initiation of eculizumab therapy, the frequency of abdominal pain attacks decreased, hemoglobin level normalized, and platelet values increased slightly. In patients submitting with a triad of symptoms such as thrombocytopenia, iron deficiency anemia, and abdominal pain attacks of unknown etiology we suggest considering PNH. We also encourage physicians to share their similar observations in order to raise the knowledge on infrequent presentations of PNH

FAS/FASL gene polymorphisms in Turkish patients with chronic myeloproliferative disorders

Introduction : Chronic myeloproliferative disorders (CMPD) are chronic myeloid hematological disorders, characterized by increased myeloid cell proliferation and fibrosis. Impaired apoptotic mechanisms, increased cell proliferation, uncontrolled hematopoietic cell proliferation and myeloaccumulation may contribute to the pathogenesis of CMPD. The aim of our study was to show the possible role of FAS/FASL gene polymorphisms in CMPD pathogenesis and investigate the association with clinical parameters and susceptibility to disease. Material and methods : We included 101 (34 polycythemia vera (PV), 23 primary myelofibrosis (PMF), 44 essential thrombocythemia (ET)) CMPD patients diagnosed according to the WHO classification criteria and 95 healthy controls in this study. All the patients and the controls were investigated for FAS/FASL gene expression, allele frequencies and phenotype features, and also FAS mRNA levels were analyzed. Results : Chronic myeloproliferative disorders patients showed increased FAS-670AG + GG genotype distribution compared with the control group (p G gene polymorphism and some clinical parameters such as splenomegaly and thrombosis (p > 0.05). No statistically significant difference in FASL+843C>T genotype or allele frequency was found between groups (p > 0.05). Moreover, no statistically significant difference was detected in FASL and JAK2V617F mutations (p > 0.05). FAS mRNA expression was 1.5-fold reduced in patients compared to healthy subjects. Conclusions : According to our findings, FAS/FASL gene expression may contribute to the molecular and immunological pathogenesis of CMPD. More investigations are needed to support these data

Prospective registry of adult patients receiving therapeutic plasma exchange with a presumptive diagnosis of thrombotic microangiopathy (TMA): The Turkish hematology research and education group (ThREG)-TMA02 study

To understand who uses social media and how often they do so, we examined the personality traits that predict frequency of using a set of 10 different types of social media (e.g., social networks, blogs, virtual worlds). Using survey data collected from two large-scale samples of young adults (Exploratory Study 1 N = 1586, Confirmatory Study 2 N =1432), we conducted direct replications of our findings to test whether the observed relationships between social media use and personality traits were reliable. Our replicated findings reveal that sex, age and extraversion are reliable predictors of several types of social media use (e.g., Facebook, Messaging Platforms, Online Forums), while other traits (e.g., agreeableness, Machiavellianism) showed unreliable associations. Our findings also reveal dimensions of multiplatform social media use that categorize the media ecologies of young adults and show significant associations with individual difference measures. (C) 2020 Published by Elsevier Inc