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Öğe Castleman Disease: A Multicenter Case Series from Turkey(Galenos Publ House, 2022) Gunduz, Eren; Kirkizlar, Hakki Onur; Umit, Elif Gulsum; Gulsaran, Sedanur Karaman; Ozkocaman, Vildan; Ozkalemkas, Fahir; Candar, OmerObjective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.Öğe CD5 as a prognostic marker in patients with diffuse large B-cell lymphoma: a multicenter study(Springer Heidelberg, 2022) Demirci, Ufuk; Kirkizlar, Hakki Onur; Umit, Elif G.; Gursoy, Vildan; Pinar, Ibrahim Ethem; Ozkalemkas, Fahir; Guven, Zeynep TugbaDiffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma (NHL) and comprises a heterogeneous group of disease. While classification of B-cell lymphomas has been evolving to include clonality in a specific manner, morphology, and immunohistochemistry remain the backbone. We aimed to evaluate the value of CD5 expression on disease characteristics as well as prognosis in patients with DLBCL. Data of 131 patients with DLBCL with CD5 positivity and as a comparison arm, data of 129 patients with DLBCL without CD5 positivity were evaluated. Mean age was 59 and 55.7% of the patients were male. Overall survival was 29.8 months. Poor prognostic factors including (high-LDH levels, B symptoms, low ECOG score, high R-IPI and NCCN-IPI score) were observed to be significantly related with CD5 positivity. Mean survival in CD5 positive patients were 29.8 months, which is significantly shorter than the general DLBCL survival worldwide. CD5 expression shall be evaluated in all samples of DLBCL patients due to its possible effects on outcomes.Öğe Comparison of Clinical Scoring Systems in the Management of Patients with Microangiopathic Hemolytic Anemia and Thrombocytopenia(Galenos Yayincilik, 2021) Baysal, Mehmet; Umit, Elif; Kirkizlar, Hakki Onur; Demir, Ahmet MuzafferObjective: Several clinical scoring systems have been developed for the differential diagnosis of thrombotic microangiopathies (TMAs), all to predict and identify patients with ADAMTS13 deficiency and to start treatment as soon as possible. The first scoring system in this regard was the Bentley score, and the French score and PLASMIC score were developed afterwards. Materials and Methods: We aimed to evaluate the laboratory parameters and clinical features of patients who underwent plasma exchange with a prediagnosis of TTP at our clinic between 2007 and 2019 and whose ADAMTS13 enzyme levels were measured and to compare the findings with the scoring systems. Results: Data of 35 patients were evaluated. Twelve patients were evaluated as high risk according to all three scoring systems. A statistically significant relation was observed between all three scoring systems and ADAMTS13 levels. Conclusion: A moderate correlation was found between all three scoring systems and ADAMTS13 levels. We observed similar potential strength of all three scoring systems to predict TTP among other TMAs and we conclude that they are applicable in daily practice.Öğe Concepts of Double Hit and Triple Hit Disease in Multiple Myeloma, Entity and Prognostic Significance(Nature Portfolio, 2020) Baysal, Mehmet; Demirci, Ufuk; Umit, Elif; Kirkizlar, Hakki Onur; Atli, Emine Ikbal; Gurkan, Hakan; Gulsaran, Sedanur KaramanRisk assessment in newly diagnosed multiple myeloma patients (NDMM) is the first and the most crucial determinant of treatment. With the utilization of FISH analysis as a part of routine practice, high risk Multiple Myeloma (MM) is defined as having at least one of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p. M-Smart MM risk stratification guideline by Mayo Clinic has proposed a concept similar to high grade lymphomas. Having two of the high risk genetic abnormalities were defined as double hit MM and having any three as triple hit MM. Based on these definitions which may bring a much more clinically relatable understanding in MM prognosis, we aimed to assess our database regarding these two concepts and their probable significance in terms of outcome and prognosis. We retrospectively evaluated 159 newly diagnosed multiple myeloma patients and their clinical course. Among these patients; twenty-four patients have one high risk determinant and also seven and two patients were classified as double hit MM and triple hit MM respectively. Overall survival (OS) of the patients with double hit MM was 6 months, 32.0 months for patients with single high risk abnormality and 57.0 months for patients with no high risk abnormality. Univariate analysis showed that Double Hit and Triple Hit MM is a predictive of low OS. Hazard Ratio of patients with one high risk abnormality was 1.42, double-hit MM patients was 5.55, and triple-hit MM patients was 7.3. Despite the development of novel drugs and their effects of prolonging survival, the treatment has not been individualized. Understanding the biology of each patient as a unique process will be the success of the treatment. As it is known that some MM patients harbor high risk genetic abnormalities according to FISH analysis, we can continue the argument that some patients bring an even higher risk and that can be defined as double or triple hit MM.Öğe Could Antinuclear Antibody Positivity Be a Factor Affecting Treatment Response in Immune Thrombocytopenia Patients on Eltrombopag?(Galenos Yayincilik, 2022) Baysal, Mehmet; Bas, Volkan; Umit, Elif; Kirkizlar, Hakki Onur; Demir, Ahmet MuzafferObjective: Eltrombopag remains a prominent option in the treatment of steroid-dependent or steroid-refractory immune thrombocytopenia (ITP) patients. Unfortunately, not all patients respond to eltrombopag. Antinuclear antibody (ANA) positivity can be seen at rates of up to 30% in ITP patients. Despite being widely used, more markers to predict the response to eltrombopag are still needed. In the present study, we aimed to show the association between ANA positivity and eltrombopag response in ITP patients. Materials and Methods: Patients who were diagnosed with ITP in the Trakya University Faculty of Medicine's Department of Hematology and who underwent eltrombopag treatment due to their resistance to steroids and other treatments were included in our study. ANA measurement was performed by indirect fluorescent antibody method and titers of 1:160 and above were considered positive. ANA measurements were made before starting eltrombopag. Results: Forty-five patients were included in our study, 33 being women and 12 men. The mean age of the patients was 45.73 years. There were 14 patients with ANA positivity and 31 patients were found to be ANA-negative. Response rates were higher in ANA-negative patients compared to ANA-positive patients in the 1st and 6th months of eltrombopag treatment (p<0.05). Conclusion: ANA positivity in ITP may indicate unresponsiveness to eltrombopag treatment, a finding that should be further supported by prospective studies involving more patients.Öğe Economic analysis of oral posaconazole prophylaxis in acute myeloid leukemia during remission induction in Turkey(Kare Publ, 2020) Umit, Elif Gulsum; Baysal, Mehmet; Kirkizlar, Hakki Onur; Demir, Ahmet MuzafferOBJECTIVE: Invasive fungal infections (IFI) are important and trending causes of mortality in patients with acute leukemia, especially during the remission induction. METHODS: In this study, 225 patients who were diagnosed with acute myeloid leukemia (AML) and undergoing intensive treatment for remission induction were enrolled in a retrospective manner. RESULTS: Within the whole group, which consisted of 225 patients, 90 patients received prophylactic antifungal treatment (PAT) (40%), while 135 patients did not (60%) receive. The mean cost of hospitalization was 9.151,6 (2.872,6-20.483,3) US dollars. Gender distribution and mean ages of groups were similar. One hundred fourteen patients not on PAT (84.4%) and five patients on PAT (5.5%) received intravenous antifungal treatment. Thirty-two of the patients who were not on PAT (23.7%) and 11 of the patients on PAT died during remission induction (12.22%). The mean day of the hospitalization was 22.61 days for the patients on PAT and 33.89 days for the patients who were not on PAT. In patients on PAT, the mean number of transfused platelet units was six (0-9), while 12.51 (4-43) units for patients who were not on PAT. CONCLUSION: In our study, the oral suspension form of posaconazole was observed to be cost-effective to prevent IFI with a significant decrease in mortality during remission induction treatment.Öğe The Importance of Targeted Next-Generation Sequencing Usage in Cytogenetically Normal Myeloid Malignancies(Mattioli 1885, 2021) Atli, Emine Ikbal; Gurkan, Hakan; Atli, Engin; Kirkizlar, Hakki Onur; Yalcintepe, Sinem; Demir, Selma; Demirci, UfukAdvanced diagnostic methods give an advantage for the identification of abnormalities in myeloid malignancies. Various researchers have shown the potential importance of genetic tests before the disease's onset and in remission. Large testing panels prevent false-negative results in myeloid malignancies. However, the critical question is how the results of conventional cytogenetic and molecular cytogenetic techniques can be merged with NGS technologies. In this paper, we drew an algorithm for the evaluation of myeloid malignancies. To evaluate genetic abnormalities, we performed cytogenetics, molecular cytogenetics, and NGS testing in myeloid malignancies. In this study, we analyzed 100 patients admitted to the Medical Genetics Laboratory with different myeloid malignancies. We highlighted the possible diagnostic algorithm for cytogenetically normal cases. We applied NGS 141 gene panel for cytogenetically normal patients, and we detected two or more pathogenic variations in 61 out of 100 patients (61%). NGS's pathogenic variation detection rate varies in disease groups: they were present in 85% of A.M.L. and 23% of M. D.S. Here, we identified 24 novel variations out of total pathogenic variations in myeloid malignancies. A total of 18 novel variations were identified in A.M.L., and 6 novel variations were identified in M.D.S. Despite long turnaround times, conventional techniques are still a golden standard for myeloid malignancies but sometimes cryptic gene fusions or complex abnormalities cannot be easily identified by conventional techniques. In these conditions, advanced technologies like NGS are highly recommended.Öğe Management of Patients with Factor VII Deficiency in Surgery: A Single-center Experience(Erciyes Univ Sch Medicine, 2022) Demirci, Ufuk; Gulsaran, Sedanur Karaman; Bas, Volkan; Umit, Elif G.; Kirkizlar, Hakki Onur; Demir, Ahmet MuzafferFactor VII deficiency is a rare condition characterized by a broad spectrum of clinical phenotypes, from asymptomatic status to severe, life-threatening bleeding, such as central nervous system or gastrointestinal bleeding. Factor VII deficiency is usually diagnosed after a bleeding attack or as a result of screening tests performed in cases with a family history. Importantly, bleeding may also occur in patients with a factor level of 20% to 50%. This report describes the approach to management before, during, and after surgery used in 5 patients with varying factor levels. The use of recombinant factor VIIa (15-30 mu g kg) was required in 3 of the patients. No bleeding or thromboembolism was observed in any of the 5 patients.Öğe Sarcopenia in Hodgkin's lymphoma evaluated with 18-FDG PET/CT, focus on age, performance, and treatment(Springer, 2021) Bas, Volkan; Umit, Elif Gulsum; Korkmaz, Ulku; Baysal, Mehmet; Karaman Gulsaran, Sedanur; Demirci, Ufuk; Kirkizlar, Hakki OnurSarcopenia is defined as a progressive and generalized muscle disorder associated with certain physiological and pathological conditions. We aimed to evaluate the prevalence of sarcopenia in patients with HL using 18-fluoro deoxyglucose (FDG) PET/CT, which would provide a data of muscle mass with the CT compartment and also data of muscle metabolism with the 18-FDG compartment of the imaging modality. Fifty-nine patients diagnosed with HL were included in the study. PET/CT images before and after treatment were evaluated with regard to lumbar muscle mass and metabolism. Mean lumbar muscle evaluation with CT before treatment was 92, 40 HU, and after treatment was 89, 41 HU. Mean metabolic tumor volume (MTV) evaluated with FDG PET before treatment was 4, 13 mm(3)while after treatment was 4, 10 mm(3). The lumbar muscle mass in terms of HU which was evaluated with CT was observed to be decreased after treatment. Likewise, the metabolic evaluation was observed to be also decreased after treatment. Despite the decline in muscle mass after treatment in the whole group, this decline was particularly observed in the better initial performance group. In patients with BMI > 32, there was a significant decline in muscle mass. Abdominal nodal involvement was related with poorer muscle mass and quality. In HL care, particular attention should be given to patients who are younger and with better physical condition in terms of preserving the muscle reserves and preventing sarcopenia.Öğe Splanchnic Venous Thrombosis, with Spotlight on Occult Malignancies, Anticoagulation, and Bleeding(Aves Press Ltd, 2018) Umit, Elif Gulsum; Baysal, Mehmet; Kirkizlar, Hakki Onur; Demir, Ahmet MuzafferObjective: Splanchnic venous thrombosis (SVT) conceptually embraces thrombosis in the portal, hepatic, splenic, and mesenteric venous system thrombosis. The SVT risk factors may be classified as abdominal disorders, underlying myeloproliferative neoplasms (MPN), inherited thrombophilic syndromes, and autoimmune disorders. The aim of our study is to evaluate the risk factors for SVT and their relations with the localization of involvement and anticoagulation during the acute period and relation to major bleeding. Methods: All patients with portal vein thrombosis or splenic venous thrombosis in their radiologic evaluation report were included over a 5-year period. Results: Of the 96 patients, 87 had an identifiable risk factor for SVT (90.6%). The major risk factor was cirrhosis (60 patients, 62.5%). Other risk factors included thrombophilic conditions (12 patients, 12.6%), 6 patients had the myeloproliferative disorder (6.3%), and most interestingly, 24 had occult malignancy for which SVT was the presenting factor (25%). Within the whole group, 51 patients (53.1%) received anticoagulant treatment. Within the whole group, 30 patients developed major bleeding (31.3%), and 20 of these patients did not receive anticoagulation therapy. Twenty-five of the patients with cirrhosis suffered bleeding, and 18 of them did not receive anticoagulation therapy. Conclusion: Almost all patients with SVT had an identifiable risk factor. The follow-up and further treatments should be based on this risk factor. SVT may be the presenting finding of occult malignancies and occult malignancy should be investigated in every patient with SVT. Anticoagulation during the initial acute period should not be withheld, even in patients with the chronic liver disease with a concern for major bleeding.Öğe Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia: Effects on Epistaxis Severity Score and Quality of Life(Galenos Yayincilik, 2019) Baysal, Mehmet; Umit, Elif G.; Kirkizlar, Hakki Onur; Ozdover, Ali Caner; Demir, Ahmet MuzafferHereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominantly inherited disorder characterized by bleeding episodes. These episodes tend to happen spontaneously and reduce the quality of life. Patients are often unresponsive to local measures. With the pathophysiological role of angiogenesis in HHT, antiangiogenic drugs including thalidomide are used to control bleeding episodes. In our study, we evaluated 6 patients with HHT, calculating their Epistaxis Severity Score (ESS) and performing a quality of life assessment with the 36-Item Short Form Health Survey Questionnaire (SF-36), and we studied the alterations of these evaluations with thalidomide treatment. Three patients were male and three were female. Mean age was 60.50 years. No side effects were observed during the treatment period. Improvements of certain SF-36 dimensions including physical functioning, physical component summary, and mental component summary and of the ESS were observed after treatment. Thalidomide may be effective to control bleeding episodes with a reasonable tolerance profile in patients with HHT.
