Elif Gülsüm Ümit

Is JAK2V617F Mutation the Only Factor for Thrombosis in Philadelphia-Negative Chronic Myeloproliferative Neoplasms?

The most common genetic disorder in Philadelphia negative chronic myeloproliferative neoplasms is the JAK2-V617F mutation. In the present study, we aimed to determine risk factors for thrombosis in patients with essential thrombocytosis and polycythemia vera. We screened the medical records of 101 patients. Risk factors which may predict thrombosis were recorded. Venous thrombosis (VT) before diagnosis was significantly higher in JAK2 positive patients. VT after diagnosis was similar in JAK2 positive and negative groups, and was significantly higher in elderly patients. Treatment places importance on the JAK2 mutation under unmodifiable cardiovascular risk factors such as advanced age after diagnosis.

Multiple Liver and Muscle Abscesses and Sepsis with Bacillus pantothenticus in a Leukemia Patient.

Received/Geliş tarihi : September 14, 2013 Accepted/Kabul tarihi : December 16, 2013 To the Editor, Bacilli infections in patients with hematological disorders have been reported, although rare, and subtypes such as Bacillus pantothenticus have been reported in immunocompetent patients with liver abscess [1]. Here we present a case of acute myeloid leukemia and multiple liver and muscle abscesses and sepsis with B. pantothenticus during the neutropenic period, treated successfully with meropenem. Informed consent was obtained. A 49-year-old male patient diagnosed with acute myeloid leukemia was started on remission induction chemotherapy with cytosine arabinoside and idarubicin (7+3). On day 8 of treatment, he had severe neutropenia (40/mm3) and fever (40.0 °C). His physical examination was normal, without organomegaly or tenderness, revealing no hints regarding the source of infection. According to the American Society of Clinical Oncology guidelines for febrile neutropenia, cefepime was started empirically at 2 g 3 times daily after blood and urine samples for culture had been obtained. After 48 h, the patient still had fever (38.3 to 39.4 °C) as tested 6 times a day. The antibiotherapy was switched to meropenem at 1 g 3 times daily following a second set of samples for culture. Fever persisted despite 48 h of meropenem treatment. Serum galactomannan antigen was negative. Thorax computerized tomography revealed no lung infection but multiple masses were seen in the liver (the largest being 3 cm). Magnetic resonance imaging indicated multiple liver abscesses with central necrosis and peripheral contrast enhancement with a single focus of abscess localized in the right paravertebral muscle groups with similar properties (Figure 1).

Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia, Effects on Epistaxis Severity Score and Quality of Life

Address for Correspondence/Yazışma Adresi: Mehmet BAYSAL, M.D., Trakya University Faculty of Medicine, Department of Hematology, Edirne, Turkey Phone : +90 284 235 76 41/2687 E-mail : drmehmetbaysal@gmail.com ORCID-ID: orcid.org/0000-0001-7681-4623 Received/Geliş tarihi: June 02, 2018 Accepted/Kabul tarihi: June 08, 2018 ©Copyright 2019 by Turkish Society of Hematology Turkish Journal of Hematology, Published by Galenos Publishing House Introduction Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder characterized by telangiectasia and arteriovenous malformations of the skin, mucosal tissues, and internal organs including the gastrointestinal tract, liver, and lungs. Its prevalence is estimated to be between 1/5000 and 1/10,000 worldwide. Recurrent epistaxis due to nasal telangiectasia is the most common finding [1,2]. There are several mutations linked with the disease and the most frequent mutations are reported as the ENG gene encoding endoglin and the ACVRL1 gene encoding activin A receptor type II-like kinase 1 [3]. Anemia is a very common symptom in HHT patients, not only due to bleeding from telangiectases located in the nasal mucosa but also to telangiectases located in the gastrointestinal tract, especially active in older ages. The diagnostic criteria for HHT were defined in 2000 and updated in 2011, including epistaxis, telangiectases, vascular malformations, and family history. The presence of 3 of these criteria is suggested to be sufficient for diagnosis [4]. Since epistaxis is the most common

Variations in Mean Platelet Volume in Patients with Helicobacter pylori Infection before and after Eradication, Way before Immune Thrombocytopenia?

Helicobacter pylori (H. pylori) and immune thrombocytopenia (ITP) association is well known and eradication treatment has its place in both treatment guidelines. Since H. pylori eradication is followed by an increase in platelet counts in patients with immune thrombocytopenia, it is suggested that H. pylori be examined and treated if infection is present. There is only one study that demonstrated a relation between H. pylori and platelet indices in individuals with normal platelet counts. In this study, we aimed to investigate the effects of H. pylori infection on platelet count and mean platelet volume, which is a sign of increased platelet destruction in patients with normal platelet counts. We evaluated the data of 106 patients with urease test positivity before the eradication of bacteria and urea breath test negative after the eradication, in a retrospective manner. Mean platelet count in patients before the eradication treatment was 256.730 ± 66.380/mm3. After H. pylori was eradicated, it has been observed that the mean platelet count increased to 287.080 ± 59.240/mm3. Mean MPV of patients before and after eradication treatment were 9.35 ± 1.63 fL and 8.61 ± 1.48 fL. Mean MPV was higher when patients were infected with H. pylori. This study showed that there is an increase in platelet counts and a decrease in MPV levels with the eradication of the H. pylori. Our study is the first to investigate changes of mean platelet volume and platelet count before and after eradication of H. pylori infection in individuals with normal platelet counts.

Drug Induced Thrombotic Microangiopathy with Certolizumab Pegol

Background: Certolizumab pegol is used to treat ankylosing spondylitis, Crohn’s disease, psoriatic arthritis, and rheumatoid arthritis. Unlike other monoclonal antibodies such as infliximab and adalimumab, certolizumab does not contain an Fc fraction and hence does not induce complement activation. In this report, we describe the case of a patient with thrombotic microangiopathy caused due to certolizumab pegol, with a brief description about the pathophysiological approach to thrombotic microangiopathy. Case Report: A-39-year-old man suffering from ankylosing spondylitis for the past 10 years presented with fatigue. He had been on certolizumab pegol treatment for 6 months, starting with 400 and 200 mg every 2 weeks. He had significant nonimmune hemolytic anemia and thrombocytopenia without a disseminated intravascular coagulopathy. Schistocytes were observed in more than 10% of the erythrocytes per field. Plasma exchange along with corticosteroid treatment was started. There was a dramatic improvement within a week, and after 10 sessions of plasma exchange, the patient was discharged on corticosteroids with a tapering plan. ADAMTS13 enzyme activity was determined to be normal. Conclusion: The development of drug-induced thrombotic microangiopathy may be either immune-mediated or dose-dependent toxicity-mediated Anti-drug antibodies and their immunological aspects are still unclear and yet to be elucidated.

Hypogammaglobulinemia and Poor Performance Status are Predisposing Factors for Vancomycin Resistant Enterococcus Colonization in patients with Hematological Malignancies.

Objective: Vancomycin-resistant enterococci (VRE) are common pathogens of hospital-acquired infection. Long hospitalization periods, use of broad-spectrum antibiotics, and immunosuppression are major risks for VRE colonization. We aimed to evaluate patients’ characteristics and factors that may contribute to VRE colonization. Materials and Methods: Data of 66 patients with colonization and 112 patients without colonization who were hospitalized in the hematology clinic were collected. Hematological malignancies, preexisting gastrointestinal complaints, the presence of hypogammaglobulinemia at the time of diagnosis, complications like neutropenic enterocolitis (NEC), and Eastern Cooperative Oncology Group (ECOG) and Karnofsky performance statuses were recorded. Results: Ages of the patients ranged between 19 and 95 years (mean: 55.99). Karnofsky and ECOG scores were statistically related to VRE colonization (p<0.000 and p<0.000), though only the Karnofsky score was significant based on logistic regression analysis. Almost all patients with acute leukemia (45 patients) had been on antibiotics (piperacillin-tazobactam, ceftazidime, and meropenem), while no patients with myelodysplastic syndrome, myeloma, or benign diseases and 2 patients with lymphoma and 1 with chronic myeloid leukemia were on antibiotics. Median time for colonization regardless of antibiotic use and diagnosis was 4.5 days (range: 3-11 days). In the VRE-colonized group, 40.9% of patients had NEC development, while in the non-colonized group, only 1.7% had NEC development. In the VRE-colonized group 46 patients (69.7%) and in the non-colonized group 27 patients (24.1%) had hypogammaglobulinemia at diagnosis; among these patients, 23 patients in the VRE-colonized group (50%) had a B-cell malignancy (lymphoma, myeloma, or chronic lymphocytic leukemia). Conclusion: Besides already anticipated diseases like leukemia, B-cell malignancies are also at high risk for colonization. This proclivity may be attributed to lack of gastrointestinal IgA due to hypogammaglobulinemia. Prolonged hospitalization (>7 days) may also be accepted as a risk factor, independent of diagnosis or antibiotic use. Performance status is also an important factor for colonization, which may be related to poorer hygiene and increased external help.

Multiple Myeloma and Thromboembolism in the Perspective of Age andPerformance

Introduction: The relation with cancer and thromboembolism (TE) are well documented. Within cancer types, hematological malignancies, especially Multiple Myeloma (MM) show a propensity towards TE with its disease biology, disease burden and treatments. We aimed to evaluate the risk factors of TE and MM with a perspective of age and clinical performance. Methods: Data regarding Eastern Cooperative Oncology Group (ECOG) and Karnofsky performance scores, patient, disease and treatment related properties of patients with MM between 2010 and 2016 were recorded. Results: Of the 125 MM patients, 60 were female (48%) while 65 were male (52%). Median age was 65 years. TE was observed in 28 patients (22.4%). In patients <65 years, poor ECOG and Karnofsky scores were strongly related with TE (p values 0.003 and 0.000). Polypharmacy and LDH elevation were observed to be risk factors in all ages (p values 0.002 and 0.000). In patients with poor ECOG (p=0.005 and 0.037) and Karnofsky performance (p=0.002 and 0.003) while radiotherapy and pneumonia during TE episodes were observed to be risk factors for TE regardless of age and performance (p values 0.016 and 0.000). Antimicrobial use during TE episode was observed to be a risk factor in younger patients (p=0.000) who are fit by both scales while bed rest and presence of fractures were observed as risk factors in younger patients with poor performance scores. Conclusion: Performance assessment should be considered as fundamental for TE evaluation and adequate prophylactic treatment for TE should be commenced in frail young patients.

CD11c expression in chronic lymphocytic leukemia revisited, related with complications and survival

Chronic lymphocytic leukemia (CLL) is a disorder of mature but dysfunctional monoclonal B cells. Microenvironment, antigenic stimulation and genetical mutations are demonstrated in etiopathogenesis. We aimed to evaluate the expression of CD11c in patients with CLL and its possible clinical significance.

Could the mosaic pattern of chromosomal abnormality predict overall survival of patients with myelodysplastic syndrome

OBJECTIVE/BACKGROUND Myelodysplastic syndromes (MDSs) are a group of monoclonal hematopoietic diseases consisting of a number of various entities. The presence of differences in chromosomal content of cells within the same individual is known as chromosomal mosaicism. The impact of mosaic pattern on the prognosis of MDS has been unclear. In this study, we aimed to determine the impact of mosaic pattern on the survival of patients with MDS. METHODS We retrospectively evaluated 119 patients diagnosed with MDS at the Trakya University Faculty of Medicine, Department of Hematology. Giemsa-Trypsin-Giemsa banding was used to evaluate chromosomal abnormality. The effect of chromosomal abnormality mosaicism on overall survival and transformation to acute leukemia was evaluated by Kaplan-Meier survival analysis. RESULTS The mean age at diagnosis was 66.3years, and the mean disease duration was 24.2months. Chromosomal abnormality was observed in 32.5% of patients. Patients with chromosomal abnormalities comprising at least 50% metaphases had significantly lower overall survival than patients with abnormality comprising up to 50% of all abnormal metaphases (p=.003). There were no differences in transformation to acute leukemia among patients with higher and lower chromosomal mosaicism (p=.056). CONCLUSION The most important outcome of this study was to demonstrate worse overall survival rates in MDS patients with higher abnormal chromosomal mosaicism than patients with lesser abnormal chromosomal mosaicism. Higher levels of abnormal chromosomal mosaicism did not predict transformation to acute leukemia. The cause of worse outcomes of patients with higher abnormal chromosomal mosaicism may be related to clonal mass.