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Objective: Hodgkin’s lymphoma has a good prognosis unless it has relapsed or become refractory. The predictive value of platelet (Plt)-related parameters, namely, mean Plt volume (MPV), plateletcrit (PCT), Plt distribution width, and Plt, is shown in some solid tumors and hematological malignancies, but it remains unknown in Hodgkin’s lymphoma. This study aimed to define their values and effects on staging and relapsing status in patients with Hodgkin’s lymphoma by comparing them with those in healthy subjects. Methods: Values of Plt-related parameters of 217 patients with Hodgkin’s lymphoma and 205 healthy individuals were documented and compared according to the disease stage and relapsing status. We defined the cutoff values for diagnosis, staging, and relapsing status of these parameters using the receiving operating characteristic curve analysis. Results: For diagnosis, the cutoff values of MPV, Plt, and PCT were 8.49 fL, 32,1000/mm³, and 0.31, respectively. For staging, the cutoff values of MPV and Plt were 9.5 fL and 12 fL, respectively. None of the parameters were associated with relapsing status. Conclusion: This is the first study evaluating Plt-related parameters in Hodgkin’s lymphoma. Further studies including survival analyses will clarify the effect of these parameters on Hodgkin’s lymphoma.

Tam Metin / Full Text Q4 SCI-Expanded WOS:000668244900016 PMID: 33315343 The aim of this study is to assess the efficacy and safety of ruxolitinib in patients with myelofibrosis. Materials and methods: From 15 centers, 176 patients (53.4% male, 46.6% female) were retrospectively evaluated. Results: The median age at ruxolitinib initiation was 62 (28–87) and 100 (56.8%) of all were diagnosed as PMF. Constitutional symptoms were observed in 84.7%. The median initiation dose of ruxolitinib was 30 mg (10–40). Dose change was made in 69 (39.2%) patients. Forty seven (35.6%) and 20 (15.2%) of 132 patients had hematological and nonhematological adverse events, respectively. The mean spleen sizes before and after ruxolitinib treatment were 219.67 ± 46.79 mm versus 199.49 ± 40.95 mm, respectively (p < 0.001). There was no correlation between baseline features and subsequent spleen response. Overall survival at 1-year was 89.5% and the median follow up was 10 (1–55) months. We could not show any relationship between survival and reduction in spleen size (p = 0.73). Conclusion: We found ruxolitinib to be safe, well tolerated, and effective in real-life clinical practice in Turkey. Ruxolitinib dose titration can provide better responses in terms of not only clinical benefit but also for long term of ruxolitinib treatment

Rosai-Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment. © 2020 Volkan Karakuş et al. The authors would like to thank Simon Edward Mumford, MSc. TESOL, Writing Centre Advisor at the Izmir University of Economics, for his great contribution in editing the language of the manuscript.

Aim: The aim of our study is to obtain data on the quality of life (QoL) in Hodgkin lymphoma (HL) patients in a representative sample of the general population of Turkey with the help of the EORTC QLQ-C30 and QLQ-HL27 questionnaires. Material and Methods: A total of 68 patients from seven different centers diagnosed with HL between 2018-2020 were included in the study. The questionnaires were answered cross-sectionally by the patient under the control of a physician in the centers participating in the study. Results: Out of 68 patients, 42.6% (n=29) were female and 57.4% (n=39) were male. The ages of the patients ranged from 18 to 74 years, with a mean of 42.10±16.62 and with a median value of 40 years. There was no significant difference between age subgroups in terms of QLQ-C30 global health status/ QoL, functional or symptom scales and HL27 SB, PC, EI and WF scores (p>0.05, for all). It was determined that the constipation scores of females were higher than the scores of males (p=0.041). No statistically significant difference was found in terms of HL27 SB, PC, EI and WF sub-dimension scores according to gender (p>0.05). Conclusions: There was only a statistically significant difference in terms of QLQ-C30 constipation sub-dimension scores according to gender. The constipation scores of females were higher than the scores of men. More detailed and large population studies are needed to reveal the effectiveness of QoL assessment in HL patients. Amaç: Çalışmamızın amacı, EORTC QLQ-C30 ve QLQ-HL27 anketleri yardımıyla Türkiye genelini temsil eden bir örneklemde Hodgkin lenfoma (HL) hastalarında yaşam kalitesi hakkında veri elde etmekti. Gereç ve yöntemler: 2018-2020 yılları arasında, HL tanısı almış yedi farklı merkezden toplam 68 hasta çalışmaya dahil edildi. Anketler, araştırmaya katılan merkezlerde hekim kontrolünde hasta tarafından yanıtlandı. Bulgular: 68 hastanın %42.6'sı (n=29) kadın, %57.4'ü (n=39) erkekti. Hastaların yaşları 18 ile 74 arasında değişmekte olup, ortalama 42.10±16.62 ve ortanca değeri 40 idi. QLQ-C30 global sağlık durumu/ yaşam kalitesi, fonksiyonel veya semptom skalaları ve HL27 SB, PC, EI ve WF skorları açısından yaş alt grupları arasında anlamlı fark yoktu (tümü için, p>0.05). Kadınların kabızlık puanlarının erkeklere göre daha yüksek olduğu belirlendi (p=0.041). Cinsiyete göre HL27 SB, PC, EI ve WF alt puanları açısından istatistiksel olarak anlamlı fark bulunmadı (p>0.05). Sonuç: Cinsiyete göre sadece QLQ-C30 kabızlık alt puanları açısından istatistiksel olarak anlamlı bir fark vardı. Kadınların kabızlık puanları erkeklerin puanlarından daha yüksekti. HL hastalarında QoL değerlendirmesinin etkinliğini ortaya çıkarmak için daha ayrıntılı ve geniş popülasyon çalışmalarına ihtiyaç olduğu görülmektedir.

Introduction: To investigate the demographics and treatment details of the acute myeloid leukemia (AML) patients who were diagnosed and followed up in Turkey. Methods: Patients who were recorded on the database of Turkish AML Registry project were included in this study retro- spectively if they were diagnosed before 1st of Jan 2022. Demographics, patient, and disease related parameters both at the time of diagnosis and at the follow up and treatment outcomes were presented.

Acquired hemophilia A (AHA) is a rare disease caused by autoantibodies inhibiting factor VIII (FVIII) activity. Although the conditionis usually idiopathic, there may be other underlying diseases. Treatment consists of two steps: treatment of acute bleeding and immunosuppression. In this multicenter study, we aimed to demonstrate the clinical characteristics, management details, and survival of AHA patients in Turkey. Data was collected from eleven centers in Turkey. aPTT, FVIII, FVIII inhibitor, and hemoglobin (HB) levels, mixing test results, and demographics at diagnosis, treatment information, adverse events, bleeding episodes during follow-up, relapses, and outcome were analyzed. Twenty-nine patients were analyzed (58.6% female). No underlying disorder could be detected in 14 patients. The most prevalent etiologies were pregnancy, malignancy and infections. The median FVIII activity and FVIII inhibitor titer at diagnosis were 0.7% (0.0-29.4%) and 32.6 BU (0.6-135.6 BU) respectively. Bleeding was severe in 44.8% of patients. The HB value was significantly lower in patients with severe bleeding. Most of the patients (n = 25, 86.2%) had only one bleeding episode without relapse, three patients (10.3%) had two bleeding episodes, and one patient had more than three bleedings. 21 (75%) patients received hemostatic therapy. The use of recombinant FVIIa was slightly higher than activated prothrombin complex concentrate (15 versus 10 patients). Immunosuppressive treatment was initiated in 26 (93%) patients. Regimens containing steroid, cyclophosphamide, and rituximab in different combinations were the most preferred. The median follow-up period was 13 months (2-156 months). Median overall survival was 154.97 months. Four and six-year survival were 90.9 +/- 0.8% and 77.9 +/- 14.1% respectively. This is a unique study that investigated the demographic characteristics, treatment approaches, and patient survival of AHA in Turkey.

Objective: Both the length of the treatment period and the diversity of the agents used in the treatment significantly affect the quality of life (QoL) of the patients with multiple myeloma (MM). With the aid of the EORTC Quality of Life Questionnaire Consisting of 30 Questions “EORTC QLQ-C30” and the Quality of Life Questionnaire Multiple Myeloma Module “QLQ-MY20”, we aimed to obtain data on quality of life in MM patients in a representative sample of the general population of our country. Methods: One hundred sixty eight patients from 6 different centers followed between 2018-2020 were included in the study. The QLQ-C30, and the QLQ-MY20 questionnaires specific for MM patients were used and the results were reported statistically. Results: Seventy eight (46%) of the patients were female, while 90 (54%) were male. The median age was 64 (22-84). When the findings were analysed, it was found that there was a greater effect on the symptom scale compared to the functional scale. Conclusion: The importance of the treatment-related side effect management, together with the adequate administration of appropriate symptomatic treatment in holistic treatment management were emphasized as effective factors in terms of the QoL of patients with MM. Amaç: Hem tedavi süresinin uzunluğu, hem de tedavide kullanılan ajanların çeşitliliği multipl miyelomlu (MM) hastaların yaşam kalitesini (YK) önemli ölçüde etkiler. Otuz sorudan oluşan EORTC Yaşam Kalitesi Anketi “EORTC QLQ-C30” ve Yaşam Kalitesi Anketi-Multipl Miyelom Modülü “QLQ-MY20” yardımıyla MM hastalarında yaşam kalitesine ilişkin verileri elde etmeyi amaçladık. Yöntem: 2018-2020 yılları arasında takip edilen, 6 farklı merkezden 168 hasta çalışmaya dahil edildi. MM hastalarına özel QLQ-C30 ve QLQ-MY20 anketleri kullanılmış ve sonuçlar istatistiksel olarak rapor edilmiştir. Bulgular: Hastaların 78’i (%46) kadın, 90’ı (%54) erkekti. Ortanca yaş 64 (22-84) idi. Bulgular incelendiğinde semptom ölçeğinde fonksiyonel ölçeğe göre daha fazla etkinin olduğu görüldü. Sonuç: Bütüncül tedavi yönetiminde, uygun tedavinin yeterli uygulanması ile birlikte tedaviye bağlı yan etki yönetiminin önemi, MM’li hastaların yaşam kalitesi açısından etkili faktörler olarak vurgulanmıştır.

Karakus, Volkan/0000-0001-9178-2850; Demirkan, Fatih/0000-0002-1172-8668; Demircioglu, Sinan/0000-0003-1277-5105; Arat, Mutlu/0000-0003-2039-8557; Cagliyan, Gulsum Akgun/0000-0002-2073-1949; Ozturk, Hacer Berna Afacan/0000-0001-9386-7604; WOS: 000473249400014 PubMed: 31036516 Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.

Thrombotic microangiopathy(TMA) is a pathological diagnosis characterized by abnormalities of small vessels leading to microvascular thrombosis of arterioles and capillaries. The current prospective, non-interventional, multicenter (n:18) study aimed to define distribution of different TMA forms in adult Turkish patients who were referred for therapeutic plasma exchange (TPE) for a presumptive diagnosis of TMA. Patients with serum ADAMTS13 activity 10 %, normal renal function and no secondary TMA were treated as unclassified TMA. The study included a total of 97 patients (female: 60; male: 30) with a median age of 48 (18?74). Detailed evaluation at 1 month after hospital admission revealed aTTP, secondary TMA, infection/complement-associated hemolytic uremic syndrome and unclassified TMA in 32 (33 %), 33 (34 %), 26 (27 %) and 6 (6%) patients respectively. As subclassification of various TMAs will dictate specific therapy, proper diagnosis in a timely manner is of utmost clinical significance. © 2021 Elsevier Ltd, Alexion Pharmaceuticals: 100064, The present study was designed as an investigator initiated trial (IIT) and sponsored by Alexion Pharmaceuticals (Tracking number: 100064)

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal stem cell disease that manifests with chronic intravascular hemolysis, thrombosis, and bone marrow failure. Various degrees of cytopenias accompany the disease. Although laboratory and clinical findings are similar, the disease may show different courses and require different treatments. Herein, we report two different courses of PNH with similar clinical and laboratory findings.

Background and aim: Uric acid elevation has been shown to be an important risk factor for cardiovascular and cerebrobascular disease. QT dispersion (QTd) is a parameter that shows the heterogeneity of ventricular repolarization and can be calculated noninvasively from surface electrocardiography. Increased QTd has been associated with severe arrhythmia and risk of sudden death in many patients and disease groups. In this context, we aimed to investigate the effect of uric acid levels on QTd and the effects of decrease in uric acid levels on QTd. Methods: A total of 225 patients with normal renal function were included in the study; 133 of these patients were hyperuricemic (>7 mg/dL), and the remaining 72 patients were normouricemic (Group 1). The hyperuricemic patients were randomly divided into 2 groups, one group (n = 67) was given placebo (Group 2) for 4 months, and the remaining 66 patients were given allopurinol 300 mg/day (Group 3). Results: Hyperuricemic patients had higher hsCRP and QTd and lower eGFR values compared to the normouricemic control group. After 4 months of treatment, 66 patients treated with allopurinol showed a significant decrease in serum uric acid, systolic and diastolic blood pressure, and hsCRP levels, and a significant increase in eGFR. Although the QTd values in the treatment group did not decrease to the same levels as in the normouricemic control group, a statistically significant decrease was found compared to their baseline values. In hyperuricemic control and normouricemic control patients, there were no differences in the levels of uric acid, hsCRP, eGFR, systolic and diastolic blood pressure, and QTd values compared to baseline values. Conclusions: There was a significant association between elevated serum uric acid and QTd, as well as with inflammatory biomarkers. Also, patients who had received hypouricemic therapy during the follow-up period presented a significant decrease in inflammatory markers as well as QTd. This indicates the beneficial effects of decreasing uric acid levels in decreasing the risk for future major adverse events related to ventricular arrhythmias.

Venetoclax is a selective B-cell lymphoma 2 (BCL2) inhibitor, which is approved to treat elderly patients with newly diagnosed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS). A total of 60 patients with a median age of 67 years from different centers were included in the final analysis. Our real-life data support the use of venetoclax in patients with both newly diagnosed and relapsed high-risk MDS and AML. Introduction: Venetoclax is a selective B-cell lymphoma 2 (BCL2) inhibitor, which is approved to treat elderly patients with newly diagnosed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS) in combination with either low-dose cytarabine (ARA-C) or hypomethylating agents. We aimed to collect and share data among the efficacy and safety of venetoclax both as a monotherapy or in combination with other drugs used to treat high-risk MDS or AML. Materials and Methods: A total of 60 patients with a median age of 67 (30-83) years from 14 different centers were included in the final analysis. Thirty (50%) of the patients were women; 6 (10%) of the 60 patients were diagnosed with high-risk MDS and the remaining were diagnosed with AML. Results: The best objective response rate (complete remission [CR], complete remission with incomplete hematological recovery (CRi), morphological leukemia-free state [MLFS], partial response [PR]) was 35% in the entire cohort. Best responses achieved during venetoclax per patient number were as follows: 7 CR, 1 CRi, 8 MLFS, 5 PR, and stable disease. Median overall survival achieved with venetoclax was 5 months in patients who relapsed and not achieved in patients who were initially treated with venetoclax. Nearly all patients (86.7%) had experienced a grade 2 or more hematologic toxicity. Some 36.7% of these patients had received granulocyte colony stimulating factor (GCSF) support. Infection, mainly pneumonia (26.7%), was the leading nonhematologic toxicity, and fatigue, diarrhea, and skin reactions were the others reported. Conclusion: Our real-life data support the use of venetoclax in patients with both newly diagnosed and relapsed high-risk MDS and AML.

Günümüzde,tıp, cerrahi alanında görülen gelişmeler ve teknolojik olarak geliştirilenmodern ilaçların kullanımıyla beraber, geçtiğimiz yüzyılda böbrek hastalıklarıda dahil olmak üzere, ölümcül olan birçok hastalık artık tedavi edilebilirkonuma gelmiştir. Böbrek nakli, elde edilen başarılar ile beraber, hem böbrek hastalıkları için bir tedaviseçeneği haline gelmiş ve böbrek nakil oranları artış göstermiş, hem debeklenen yaşam süresi uzamıştır. Böbrek nakli yapılan hastaların cerrahisonrası tedavi süreçlerinde immünsüpresif ilaçlar kullanılmaktadır. Yenigeliştirilmeye çalışılan tedavi seçenekleri ile hem böbrek naklinde elde edilenbaşarı oranı arttırılmaya çalışılmakta hem de immünsüpresif ilaçlarınkullanımının yol açtığı yan etkilerin giderilmesi amaçlanmaktadır. Hücreseltedaviler de böbrek naklinde kullanılması için üzerinde çalışılan tedaviseçeneklerinden bir tanesidir. Regülatör T hücreler, regülatör B hücreler, regülatörmakrofajlar, dendritik hücreler, mezenkimal kök hücreler, organ nakillerindekullanılması için araştırma ve deneme yapılan hücre kaynaklarındandır. Deneyhayvanlarında nakil modellerinde yapılan çalışmalarda, hücre tedavileri ilebaşarılı sonuçlar alındığı gösterilmiştir. Bu derlemede böbrek naklindekullanılan ve de kullanılmaya aday olan hücre tedavileri tartışılmıştır., Atthe present time, with the development of medicine, surgery and the use oftechnologically advanced modern drugs, many diseases, including kidneydiseases, have become treatable. Kidneytransplantation has become a treatment option for kidney diseases and kidneytransplant rates have increased as well as life expectancy has been prolongedby means of obtained success. Immunosuppressive drugs are used in theposttransplant treatment process of renal transplant patients. With the newtreatment options, it is aimed to increase the success rate of kidneytransplantation and also to eliminate the side effects which are caused by theuse of immunosuppressive drugs. Cellular therapies are one of the treatmentoptions that are studied for use in kidney transplantation. Regulator T cells,regulator B cells, regulator macrophages, dendritic cells, mesenchymal stemcells are cell sources that are searched and experimented for use in organtransplantation. It has been shown that successful results have been obtainedby using cell therapies in studies carried out with experimental animal modelsfor transplantation. In this review, cell therapies used in and candidatefor renal transplantation are discussed.

Azik, Fatih/0000-0001-5715-4244; karakus, volkan/0000-0001-9178-2850 WOS: 000539665000001 PubMed ID: 32441176 Chronic anemia, transfusion-associated iron deposition, and chelating agents lead to renal impairment in beta-thalassemia (beta-thal) patients. The present study aimed to determine the most reliable and practical method in assessing and predicting renal injury in beta-thal major (beta-TM) patients. Therefore, we assessed the predictive values of urine beta 2-microglobulin (beta 2-MG) and neutrophil gelatinase-associated lipocalin (NGAL) levels, their ratios to urine creatinine, and serum endocan level. Sixty beta-TM patients and 30 healthy controls were included. Renal functions of the patients and controls were evaluated by means of urine protein/creatinine ratio, urine beta 2-MG, urine NGAL, and serum endocan level. The beta-TM and control groups were comparable in terms of the demographic characteristics. Of the beta-TM patients, 26.7% had glomerular hyperfiltration and 41.7% had proteinuria. Compared with the control group, the beta-TM group had significantly higher levels of urine protein/creatinine, urine beta 2-MG, urine beta 2-MG/creatinine, urine NGAL, urine NGAL/creatinine, and serum endocan. These parameters did not differ between the chelating agent subgroups in the patient group. Urine beta 2-MG/creatinine and NGAL/creatinine ratios were the parameters with high specificity in predicting proteinuria. There were significant correlations of urine beta 2-MG, urine NGAL, and serum endocan levels with serum ferritin concentration. Urine beta 2-MG/creatinine, NGAL/creatinine, and protein/creatinine ratios were correlated with each other in the patient group. Positive correlations of urine beta 2-MG, urine NGAL, and serum endocan levels with serum ferritin concentration indicated that iron deposition was associated with endothelial damage and renal injury. Mugla Sitki Kocman University Scientific Research Projects UnitMugla Sitki Kocman University [16/133] The study was supported by the Mugla Sitki Kocman University Scientific Research Projects Unit with the project number 16/133.

WOS:000650535000001 PMID: 33988739 The aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17-77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (n = 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7-183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease.

0000-0003-0238-2236; 0000-0001-9178-2850 WOS: 000440304500012 PubMed ID: 30023978 Objective: In cases presenting with lymphadenopathies (LAP) without a primary focus detected by simple radiological methods, the primary tumor can be diagnosed by a histopathological evaluation of the metastatic lymph nodes. We aimed to discuss the nonhematological malignancies presenting with lymphadenopathies and the histopathological results for primary tumors. Material and Methods: In this retrospective study, cases diagnosed with metastasis in excisional lymph nodes between January 2013 and June 2016 were assessed for a histopathological diagnostic approach Results: Among 632 lymph node biopsies, a total of 21 cases, involving 12 male and 9 female patients with a mean age of 57.23 y (range, 33-92 y), of nonhematological solid tumors were included. The most common localizations of the involved lymph nodes were inguinal (n=8), axillary (n=6), cervical (n=4), and supraclavicular (n=3) region. The most common primary tumors were malignant melanoma (n=6), breast carcinoma (n=4), ovarian carcinoma (n=2), squamous cell carcinoma (n=2), and germ cell tumor (n=2). Others were papillary thyroid carcinoma, renal cell carcinoma, urothelial carcinoma, prostate adenocarcinoma, and endometrial adenocarcinoma. Conclusion: Nonhematological malignancies presenting with lymphadenopathies are one of the most complicated cases for clinicians. The histopathological evaluation of the excisional metastatic lymph node biopsies is an important method because of cost effectiveness and easy applicability.

Aim: Pancreatic exocrine dysfunction has many causes and often results in malnutrition. The relationship between chronic renal disease related malnutrition and pancreatic exocrine function has not been understood clearly yet. There is lack of data in the literature regarding pancreas exocrine dysfunction in kidney diseases. This study aimed to show whether existence of this relationship. Material and Method: 40 End stage renal disease (ESRD) patients, 40 chronic renal disease (CKD) patients and 42 healthy volunteers without any diagnosed systemic diseases were included. ‘Mini Nutritional Assessment’ (MNA) form has been filled up to determine the nutritional status of the participants. Fecal elastase 1 (FE1) levels measured in order to evaluate the exocrine function of pancreas. Results: FE1 values of control group were significantly higher from CKD and ESRD patients both. When considering the sensitivity of FE1 to show pancreas exocrine function, these results worsen pancreas exocrine function in patients with kidney disease. Conclusion: Malnutrition in kidney diseases has various reasons, and pancreatic exocrine dysfunction thought to be one of them. FE1 levels may be used in order to evaluate the exocrine functions of the pancreas. Amaç: Kronik böbrek hastalığı (KBH) ilişkili malnutrisyon ile pankreatik ekzokrin disfonksiyon arasındaki ilişki henüz net olarak açıklanamamıştır. Böbrek hastalıklarında pankreatik ekzokrin Introduction End stage renal disease (ESRD) and chronic renal disease (CRD) are serious health care problems with increasing incidences, and 5 year-mortality rates are about 60% which is due to the disease itself and the complications. Malnutrition is supposed to be one of the leading causes that contributing reasons. Malnutrition prevalence is predicted as 10–70%, and 18–50% receiving hemodialysis (HD) and continuous ambulatory peritoneal dialysis (CAPD) therapies respectively1–4. disfonksiyona ilişkin literatür yeterli değildir. Bu çalışmanın amacı böyle bir ilişkinin olup olmadığının araştırılmasıdır. Materyal ve Metot: 40 SDBH, 40 KBH ve 42 sağlıklı gönüllünün dahil edildiği çalışmada katılımcıların beslenme durumlarının belirlenmesi amacıyla “Kısa Nütrisyonel Değerlendirme (KND)” formu doldurulmuş ve pankreasın ekzokrin işlevlerinin belirlenmesi için fekal elastaz 1 (FE1) düzeyleri ölçülmüştür. Bulgular: Kontrol grubunda FE1 düzeyleri KBH ve SDBH ggruplarına göre anlamlı derecede yüksek bulunmuştur. FE1’in pankreas ekzokrin fonksiyonunu göstermedeki duyarlılığı göz önüne alındığında, sonuçlar böbrek hastalarında kötü pankreatik ekzokrin fonksiyonunu işaret etmektedir. Sonuç: Böbrek hastalığında malnutrisyonun çok farklı nedenleri olabilmesinin yanı sıra, pankreatik ekzokrin disfonksiyon bu nedenlerden biri olarak düşünülebilir. FE1 düzeyleri pankreasın ekzokrin fonksiyonunun belirlenmesinde kullanılabilir.

Helicobacter pylori (H pylori) populasyonun %50'sinden fazla gorulen ve gastrik mukozaya yerlesen spiral sekilli, flajelli, mikroaerofilik, gram (-) bir basildir. Dunya genelinde en yuksegi gelismekte olan ulkelerde bildirilen degisken bir gorulme sikligina sahiptir. Risk faktorleri ile ilgili calismalar ozellikle sosyoekonomik faktorler uzerinde durmaktadir. Insanlarda gastrit ve ulser ile iliskisi net olarak kanitlanmistir. Enfeksiyon cocukluk caginda siklikla oral yolla bulasmaktadir. Ure nefes testi, diski antijen testi, antikor tayini, endoskopi, histolojik inceleme, ureaz testi ve kultur tanida kullanilan yontemlerdendir. Antibiyoterapi ve antiasitler tek basina yeterli olmadigindan birlikte kullanimlari tercih edilmektedir. N-asetilsistein gibi mukolitik bir ajan ile H pylori tabakasinin ortadan kaldirilmasi da tedavi oncesinde etkili olabilmektedir. Lactobacillus, Saccharomyces, Bifidobacterium ve Bifidobacterium clausii gibi probiyotik suslarin eklenmesi de diger bir tedavi yaklasimidir. Ilk tercih tedaviler yetersiz kaldiginda farkli antibiyotikleri iceren ikinci adim tedavilere gerek duyulabilmektedir

61st Annual Meeting and Exposition of the American-Society-of-Hematology (ASH) -- DEC 07-10, 2019 -- Orlando, FL, Kaynar, Leylagul/0000-0002-2035-9462; Erkurt, Mehmet Ali/0000-0002-3285-417X, WOS:000577164605146, [No Abstract Available], Amer Soc Hematol

Objective The aim of the present study was to evaluate the efficacy and safety of eltrombopag, an oral thrombopoietin receptor agonist, in patients with chronic immune thrombocytopenia (ITP). Materials and Methods A total of 285 chronic ITP patients (187 women, 65.6%; 98 men, 34.4%) followed in 55 centers were enrolled in this retrospective cohort. Response to treatment was assessed according to platelet count (/mm3) and defined as complete (platelet count of >100,000/mm3), partial (30,000-100,000/mm3 or doubling of platelet count after treatment), or unresponsive (

Thrombotic thrombocytopenic purpura (TTP) is a disease characterized by the presence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, caused by the congenital or acquired decrease of the enzyme activity which degrades unusual large vWF multimers. There is no identifiable cause in half of the acquired TTP cases. Herein, we report four possible pesticide-related cases with decreased ADAMTS13 enzyme activity, increased titer of ADAMTS13 inhibitor and typical clinical and laboratory presentation

WOS: 000482629200004, PubMed ID: 31131598, Objective: Significant developments occurred in the clinical management of acute lymphoblastic leukemia (ALL) in adults in recent decades. However, treatment results are still not satisfactory, especially in routine practice. The objective of this study was to evaluate the general clinical features, treatment details, and outcomes of a large group of patients followed in multiple centers in Turkey with a diagnosis of ALL. Materials and Methods: A retrospective analysis of the data of patients with ALL was made, the patients having been diagnosed and treated between January 2003 and June 2017 by different protocols in the hematology clinics of ten different centers. A total of 288 patients, aged between 17 and 76 years old, were included in the study. In this retrospective multicenter analysis of patients with ALL, classification of patients was performed based on treatment period, Philadelphia chromosome positivity, treatment regimen, and administration of allogeneic hematopoietic stem cell transplantation (allo-HSCT). Results: The majority of cases were B-cell in origin, while 224 patients had B-ALL and 64 of the patients had T-ALL. Median follow-up duration for all patients was 18.2 months (range: 0.03-161 months). Philadelphia chromosome positivity was determined in 49 patients (21.9%), and 54 patients (18.8%) were receiving allo-HSCT. After induction chemotherapy, 219 patients (76.0%) achieved complete remission, 32 patients (11.2%) were evaluated as treatment refractory, and 37 patients (12.8%) were deceased. Median overall survival was 47.7 months (95% confidence interval: 36.1-59.2) and median disease-free survival was 23.4 months (95% confidence interval: 6.7-40.0) for all patients. Conclusion: Multicenter studies are extremely important for defining the specific clinical features of a particular disease. The results of this study will make a significant contribution to the literature as they reflect real-life data providing valuable information about the Turkish ALL patient profile.

Acquired hemophilia A, due to spontaneous autoantibody against FVIII, is a rare hemorrhagic disorder with an incidence of about 1 per million population per year. If unrecognized and untreated, it is associated with a high morbidly and mortality rate of 8 to 12%. Autoantibody against coagulation factor VIII neutralizes procoagulant activity. We report herein is such a rare case of acquired hemophilia in a patient with CLL.

β-Thalassemia (β-thal) is a common autosomal recessive disorder resulting from over 300 different mutations of the β-globin genes. Our aim was to create a mutation map of β-thal in the province of Antalya, Turkey. In this study, mutation analysis of a total 146 of β-thal patients followed at the Thalassemia Center of the Antalya Education and Research Hospital, Antalya, Turkey, were included. Direct DNA sequence analysis was performed for mutation scanning of the β-globin gene. One hundred and forty-six patients with β-thal including all types were analyzed, and 14 different β-thal mutations were detected. The most frequently seen mutation was HBB: c.93 - 21G A [IVS-I-110 (G A)] (52.7%), followed by HBB: .c.92 + 6T C [IVS-I-6 (T C)] (14.4%), HBB: c.-80T A [-30 (T A)] (8.2%), HBB: c.315 + 1G A [IVS-II-1 (G A)] (8.2%), which made up 83.1% of the observed mutations. Our results indicate the importance of micromapping and epidemiology studies of thalassemia, which will assist in establishing the national prevention and control program in Turkey.

Amaç Hematolojik hastalıklardaki deri bulgularının sıklığı ile ilgili ülkemizden bildirilen az sayıda çalışma bulunmaktadır. Bu çalışma ile hastanemiz Hematoloji polikliniğinde takip edilen hastalardaki dermatolojik bulguların prevalansını incelemeyi amaçladık. Gereç ve Yöntem Şubat 2014-Haziran 2016 tarihleri arasında hastanemiz Hematoloji polikliniğinde takip edilen 217 hastayı içeren kesitsel bir çalışma yürütüldü. Hematolojik hastalık tanıları, hastalık süresi, takip süresi, dermatoloji konsültasyonu ile elde edilen dermatolojik muayene verileri ve diğer malignite varlığı hasta dosya kayıtlarından retrospektif olarak incelendi. Bulgular İki yüz on yedi hastanın (102 kadın, 115 erkek) yaş ortalaması 63.06±14.7 yıl bulundu. Ortalama hematolojik hastalık süresi 27.98±35.9 ay ve hastalık takip süresi ise 12.21±6.65 ay olarak saptandı. En sık görülen deri bulguları sırasıyla purpura (%95 GA, %7.4- %16.1), pruritus (%9.7; %95 GA, %6-%13.8) ve viral enfeksiyonlar (%5.1; %95 GA, %2.3-%8.3) şeklinde saptandı. Miyelodisplastik sendromlu iki hastada talidomid ve deferasirox ile ilişkili vaskülitik ilaç reaksiyonu gözlendi. Spesifik kutanöz infiltrasyonlardan, kutane lösemi kronik lenfositik lösemili bir hastada; granülositik sarkom ise akut miyeloid lösemili bir hastada saptandı. Sonuç Sıklıkla immunsuprese olan ve çoklu ilaç kullanımına sahip hematoloji hastalarında, deri bulguları oldukça sık görülmekte olup geniş bir çeşitliliğe sahiptir. Hematolojik hastalıkların deri bulguları ciddi bir enfeksiyonun, ilaç reaksiyonunun veya hastalığın deriye yayılımının göstergesi olabileceğinden, bu hastalıkların teşhis ve tedavisi prognostik öneme sahiptir. Objective There are a few studies regarding the frequency of skin findings in hematological diseases reported from our country. We aimed to investigate the prevalence of dermatological findings in patients who were followed up at the Hematology outpatient clinic of our hospital with this study. Materials and Methods A cross-sectional study involving 217 patients who were followed up at the Hematology outpatient clinic of our hospital between February 2014-June 2016 was conducted. Hematologic disease diagnoses, disease duration, follow-up period, dermatological examination data obtained with dermatology consultation, and presence of any other malignancy were retrospectively reviewed from the patient file records. Results Of 217 patients (102 female, 115 male), the mean age was 63.06±14.7 years. The mean duration of hematologic disease was 27.98±35.9 months and the mean duration of disease follow-up was 12.21±6.65 months. The most common skin findings were purpura (95% GA, 7.4%-16.1%), pruritus (9.7%, 95% GA, 6%-13.8%), and viral infections (5.1%, 95% GA, 2.3%-8.3%), respectively. In two patients with myelodysplastic syndrome, thalidomide- and deferasirox-associated vasculitic drug reactions were observed. From specific cutaneous infiltrations, cutaneous leukemia was found in a patient with chronic lymphocytic leukemia and granulocytic sarcoma in a patient with acute myeloid leukemia. Conclusion Skin findings are quite common and have a wide variety in hematologic patients with frequent immunsupression and multiple drug use. Because skin findings of hematologic diseases can be a sign of serious infection, drug reaction, or cutaneous spread of the disease, diagnosis and treatment of these diseases have prognostic significance.

61st Annual Meeting and Exposition of the American-Society-of-Hematology (ASH) -- DEC 07-10, 2019 -- Orlando, FL, Onec, Birgul/0000-0003-2824-1044; OZDOGU, HAKAN/0000-0002-8902-1283, WOS:000577164604216, [No Abstract Available], Amer Soc Hematol

Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTSI3 activity/antiADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CAHUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1-75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE. (C) 2018 Elsevier Ltd. All rights reserved.

Synchronous malignancies are rare conditions in oncology practices, generally seen as solid tumors with hematological neoplasms. However, occurrence of two different hematological malignancies in the same patient is extremely rare. Two primary malignancies should be considered especially in patients with extraordinary presentations and treatment resistance. © 2018 Polish Society of Hematology and Transfusion Medicine, Insitute of Hematology and Transfusion Medicine, Published by Sciendo 2018.

Extramedullary hematopoiesis (EMH) is a phenomenon in which hematopoietic cells are found in sites other than the bone marrow. It is usually observed in the liver and spleen but may occasionally be found within solid tumors. The current case report presents a 69-year-old female patient who presented with a renal cyst. Histopathological examination following surgical removal of the cyst revealed a lining of oncocytic- and chromophobe-type cells with capsular invasion and a mass forming EMH with evident bone trabeculae within the cyst wall. Circulating hematopoietic stem cells in the blood and their colonization within tissues is discussed in the present case report, emphasizing certain types of renal cell carcinoma.