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Introduction and Objective: The Coronavirus 2019 (COVID-19) pandemic has affected many healthcare services as well as transfusion medicine practices. In this study, it is aimed to examine the early and late effects of the pandemic on the use and destruction rates of blood and blood components. Material and method: This retrospective study was conducted at a tertiary hospital transfusion center. Unit numbers of blood and blood components used and destroyed in 2018-2021 were grouped according to the pre-pandemic period (27 months), early pandemic period (6 months), and late pandemic period (15 months). Monthly use and disposals in the early and late pandemic periods were compared with the pre-pandemic period. Results: A total of 144114 units of blood and blood components were used. The most commonly used components were erythrocyte concentrate (EC), fresh frozen plasma (FFP) and thrombocyte concentrate (TC), respectively. Their monthly usages in the pre-pandemic period, the early pandemic period and the late pandemic period were 2067.1±147.2, 840±161.3 and 285.4±77.4; 1564.5±287.5, 576.7±146.2 and 261.5±52.6; 1803.5±122.1, 718.6±118.8 and 325.7±52.1; respectively. EC and FFP were used significantly less in the early and late pandemic periods than in the pre-pandemic period (p

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.

Objective: Encapsulating peritoneal sclerosis (EPS) is a rare, but potentially fatal complication of peritoneal dialysis. Currently, treatment of peritoneal fibrosis is not fully possible yet. In this study, we aimed to demonstrate the effects of tacrolimus therapy on peritoneal fibrosis and inflammation when administered alone or with mycophenolate mofetil (MMF) in the EPS model induced in rats. Methods: Thirty six Wistar albino rats were separated into six equal groups. Group I was the control group. Group II-VI were administered intraperitoneal chlorhexidine (CH) for induced EPS model in rats. Group II, IV, V, VI were administered isotonic liquid, tacrolimus, tacrolimus and concurrently with CH, tacrolimus and MMF together, respectively. Group III was not administered any drug. All peritoneal samples were stained immunohistochemically with matrix metalloproteinase-2 (MMP-2) antibody. Thickness of peritoneal fibrosis, subserosal large collagen fibers, subserosal fibroblast proliferation and subserosal fibrotic matrix deposition were evaluated. Results: Comparing the experimentally induced EPS groups, the best histopathological results and the largest staining with MMP-2 were achieved in Group VI. Furthermore, in all treatment groups (IV, V, VI) more staining with MMP-2 was detected compared to non-treatment groups (I, II, III) but no statistically significant differences were found among all groups. A statistically significant remission was observed in all histopathological parameters, primarily peritoneal thickness in rats that were administered MMF with tacrolimus, compared to rats which were administered tacrolimus only. Conclusion: Concurrent use of tacrolimus and MMF in the treatment of EPS may be a promising approach. RESUMEN Objetivos: La esclerosis peritoneal encapsulante (EPE) es una complicación rara, peropotencialmente fatal de la diálisis peritoneal. Actualmente, el tratamiento de la fibrosis peritoneal aún no es posible. En este estudio, apuntamos a demostrar los efectos de la terapia con tacrolimus en la fibrosis peritoneal y la inflamación cuando se administran solos o con micofenolato de mofetilo (MMF) en el modelo EPE inducido en ratas. Métodos: Treinta y seis ratas Wistar albinas se separaron en seis grupos iguales. El Grupo I era el grupo de control. En los grupos II-VI se administró clorhexidina intraperitoneal (CH) para el modelo EPE inducido en ratas. En los Grupos II, IV, V, VI se administró respectivamente líquido isotónico, tacrolimus, tacrolimus y CH y finalmente tacrolimus y MMF juntos. El grupo III no recibió ningún medicamento. Todas las muestras peritoneales se tiñeron inmunohistoquímicamente con el anticuerpo Matrix Metaloproteinasa-2 (MMP- 2). Se evaluó el grosor de la fibrosis peritoneal, se evaluaron las fibras de colágeno grandes subserosas, la proliferación de fibroblastos subserosa y la deposición de la matriz fibrótica subserosa. Resultados: Comparando los grupos de EPE inducidos experimentalmente, los mejores resultados histopatológicos y la tinción con MMP- 2 más extensa se lograron en el Grupo VI. Además, en todos los grupos de tratamiento (IV, V, VI) se detectó más tinción con MMP-2 en comparación con los grupos de no tratamiento (I, II, III), pero no se encontraron diferencias estadísticamente significativas entre todos los grupos. Se observó una remisión estadísticamente significativa en todos los parámetros histopatológicos, principalmente el espesor peritoneal en ratas que recibieron MMF con tacrolimus, en comparación con las ratas que recibieron solo tacrolimus. Conclusión: El uso concurrente de tacrolimus y MMF en el tratamiento de EPS puede ser una aplicación prometedora.

Amaç: Vitamin B12 eksikliğinde gelişen ciddi megaloblastik anemili olguların parenteral yoldan tedavisi sırasında hipokalemi gelişebilir. Biz de şiddetli megaloblastik anemi tanısıyla parenteral yoldan tedavilerine başladığımız hastalarımızın serum potasyum seviyelerini ve EKG bulgularını değerlendirmeyi amaçladık. Materyal Metod: Çalışmaya 9 erkek, 11 kadın 20 hasta alındı. Ortanca yaş 66.5 yıldı. Olguların tedavi öncesi ve tedavinin üçüncü gününde serum potasyum düzeyleri ve EKG bulguları arasındaki farklar ‘bağımlı grupta t testi’ uygulanarak değerlendirildi. Normal dağılım göstermeyen değişkenler için ortanca (sıra değeri) kullanıldı. Sonuçlar: Tedavinin üçüncü gününde serum potasyum seviyesinde izlenen düşüş tedavi öncesi değerle karşılaştırıldığında anlamlı değildi, 4.56mmol/L e karşın 4.28mmol/l; p=0.062. PR aralığı tedavi öncesi ortalama 0.157±0.05sn (ortanca 155msn), tedavinin üçüncü gününde ortalama 0.167±0.02 sn (ortanca 160 msn); p=0.024 bulundu. Düzeltilmiş QT aralığı (QTc) tedavi öncesi ortalama 0.379±0.074 sn (ortanca 400 msn), tedavinin üçüncügününde ortalama 0.420±0.047sn (ortanca 400msn); p=0.890 bulundu. Tartışma: Hipokalemi, tedaviye erken yanıtta yeni hematopoietik hücrelerin üretimi nedeniyle potasyum kullanımındaki belirgin artıştan kaynaklanmaktadır. Çalışmamızda, tedavinin erken döneminde ortalama potasyum seviyesinde kısmi bir azalma ve EKG’de PR aralığında anlamlı uzama tespit etmiş olmamıza karşın klinik bulgular görülmemiştir, fakat yine de bu tür hastalar tedavi sırasında yakından izlenmelidir. Objective:WeevaluatedserumpotassiumlevelsandECGfindingsofpatientswithseveremegaloblasticanemiaundergoingparenteralvitaminB12treatmentduringwhichhypokalemiamaydevelop. MaterialandMethods:Ninemaleand11femalepatientswithmedianage66.5yearswereincludedinthestudy.SerumpotassiumlevelsandtheECGfindingswereevaluatedbeforeandonthethirddayofthetreatmentandthedifferencebetweenwasevaluatedbyt-testindependentgroups. Results:Therewasnostatisticaldifferencebetweenthemeanvaluesofserumpotassiumbeforetreatmentandafterthe3rddayofthetreatment:4.56mmol/Lvs4.28mmol/L;p=0.062.Bytheway,themeanvaluesofPRintervalandQTcwereasfollows:0.157±0.05sec(median155msec)vs0.167±0.02sec(median160msec);p=0.024and0.379±0.074sec(median400msec)vs0.420±0.047sec(median400msec);p=0.890.

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

OBJECTIVE: Acute myeloid leukemia (AML) is a hematological malignancy that frequently affects elderly population. With introducing the hypomethylating agents (HMAs) in elderly AML treatment, survival rates and quality of life have improved. However, long-term management in elderly and frail patients is still a challenge. In the present study, we aimed to determine whether HMA maintenance therapy is required until disease progression in frail and elderly AML patients by examining with a real-life data. METHODS: In a multicenter study, we analyzed non-promyelocytic elderly AML patients who were treated with first-line azacitidine or decitabine monotherapy in two different groups, retrospectively. While patients were treated with HMA until progression in the maintenance group, 6+3 cycles of azacitidine or decitabine were administered as a standard care of elderly AML patients in the non-maintenance group. Survival outcomes were compared between the groups. RESULTS: HMA therapy was maintained until progression in 20 patients, and HMA therapy was terminated after 6+3 cycles in 21 patients. Patients received a median of 6 (1–14) HMA cycles during follow-up time. The median 7.5 months of overall survival were observed (2–17 months) in maintenance and 3 months (1–13 months) in non-maintenance groups (p=0.001). CONCLUSION: Despite long-term exposure to HMA may appear as a risk factor for complications and toxicities in elderly and frail AML patients, the maintenance of therapy until disease progression provides a significant survival advantage. Therefore, we suggest that HMA therapy should continue until disease progression regardless the sort of HMA.

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.

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., 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.

Hepatorenal syndromeis a reversible acute renal impairment that occurs in patients with advancedliver cirrhosis or fulminant hepatic failure. Terlipressin is a syntheticlong-acting analogue of vasopressin that is used in the treatment ofhepatorenal syndrome. Ischemic adverse events of the treatment with terlipressinhave been reported. We present a rare complication of terlipressin usage,ischemic skin necrosis of the scrotum, in a patient with advanced livercirrhosis and hepatorenal syndrome., Hepatorenal sendrom, ilerlemiş karaciğer sirozuveya fulminan karaciğer yetmezliği olan hastalarda ortaya çıkan, tersinir birakut böbrek yetmezliğidir. Terlipressin, hepatorenal sendromun tedavisindekullanılan sentetik uzun süre etkili bir vazopressin analoğudur. Terlipressinile tedavinin iskemik advers olayları bildirilmiştir. Karaciğer sirozu vehepatorenal sendromlu bir hastada terlipressin kullanımının nadir görülen birkomplikasyonunu, skrotumun iskemik cilt nekrozunu sunuyoruz.

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).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 (/mmThe median age at diagnosis was 43.9±20.6 (range: 3-95) years and the duration of follow-up was 18.0±6.4 (range: 6-28.2) months. Overall response rate was 86.7% (n=247). Complete and partial responses were observed in 182 (63.8%) and 65 (22.8%) patients, respectively. Thirty-eight patients (13.4%) did not respond to eltrombopag treatment. For patients above 60 years old (n=68), overall response rate was 89.7% (n=61), and for those above 80 years old (n=12), overall response rate was 83% (n=10). Considering thrombocyte count before treatment, eltrombopag significantly increased platelet count at the 1Results of the current study imply that eltrombopag is an effective therapeutic option even in elderly patients with chronic ITP. However, patients must be closely monitored for response and side effects during treatment. Since both response and side effects may be variable throughout the follow-up period, patients should be evaluated dynamically, especially in terms of thrombotic risk factors.Bu çalışmanın amacı kronik immün trombositopeni (ITP) hastalarında bir oral trombopoietin reseptör agonisti olan eltrombopagın etkinlik ve güvenirliliğini değerlendirmektir.Elli beş merkezde izlem altındaki toplam 285 kronik ITP hastası (187 kadın, %65,6) bu geriye dönük küme çalışmasına alınmıştır. Tedaviye yanıt trombosit sayısına göre değerlendirilmiş ve tam yanıt (100.000/mmTanı anında yaş ortalaması 43,9±20,6 (3-95) yıl olan hastalar ortalama 18,0±6,4 (6-28,2) ay izlenmiştir. Tam ve kısmi yanıtı içeren toplam yanıt %86,7 (n=247) bulundu. Sırasıyla 182 (%63,8) ve 65 (%22,8) hastada tam ve parsiyel tedavi yanıtları gözlenmiştir. Otuz sekiz hasta (%13,4) eltrombopag tedavisine yanıt vermemiştir. Altmış yaş üzerindeki hastalarda (n=68) toplam yanıt %89,7 (n=61) bulunurken, bu oran 80 yaş üzerindeki (n=12) hastalarda %83 (n=10) olmuştur. Tedavi öncesi trombosit sayısı göz önüne alındığında, eltrombopag, tedavinin 1., 2., 3., 4. ve 8. haftalarında trombosit sayısını anlamlı şekilde artırmıştır. Kısmi veya tam cevap için gereken süre arttıkça, tedaviye cevap önemli ölçüde azaldığı saptanmıştır. Eltrombopag tedavisinden sonra maksimum trombosit sayısı ne kadar yüksekse, yan etkilerin oluşabilme ihtimalinin o kadar yüksek olabildiği dikkati çekmiştir. En sık görülen yan etkiler baş ağrısı (%21,6), güçsüzlük (%13,7) ve hepatotoksisite (%11,8) ve trombozdur (%5,9).Mevcut çalışmanın sonuçları, eltrombopag tedavisinin kronik ITP’de, yaşlı hastalar dahil olmak üzere, etkili bir tedavi seçeneği olduğunu göstermektedir. Bununla birlikte, hastalar tedavi sırasında yanıt ve yan etkiler açısından yakından izlenmelidir. Hem cevap hem de yan etkiler, takip süresi boyunca değişken olabileceğinden, hastalar özellikle tromboz risk faktörleri açısından dinamik olarak değerlendirilmelidir.

Giriş: Beta-talasemi major (?-TM) hastalarında hemosiderozise bağlı kalp yetmezliği sık görülmektedir. Kalp yetmezliğini erken dönemde tespit etmek için manyetik rezonans görüntüleme (MRI) yöntemi kullanılmaktadır. N-terminal beyin natriüretik peptid (NT-proBNP) kalp yetmezliğinin tanısında oldukça hassas bir belirteçtir. Bu çalışmamızda T2* skoruna göre kardiyak demir birikimi olmayan (CMRT2* > 20 msn) ?-TM hastalarında, kalp yetmezliğinin erken dönemde tanısında NT-proBNP'nin etkinliğini araştırdık.Yöntem: T2* > 20 ms olan 31 ?-TM hastası ve 25 sağlıklı kişide NT-ProBNP düzeyleri kemilüminesans yöntem ile ölçüldü.Bulgular: Hastaların NT-proBNP düzeyleri [ortanca: 33 (IQR: 28-94) pg/mL] ile kontrol grubu [ortanca: 41 (IQR: 28-59) pg/mL] arasında anlamlı bir fark tespit edilmedi. Hastaların 6'sında NT-proBNP düzeylerinin cut-off değerinin üzerinde olduğu belirlendi.Sonuç: MRI yöntemi ile kıyaslandığında NT-proBNP daha ucuz, ulaşılabilir ve girişimsel olmayan bir yöntemdir. Talasemi hastalarının aylık kontrollerinde dahi kolaylıkla uygulanabilir. T2* skoru normal olan hastaların bazılarında NT-proBNP düzeylerinin cut-off değerlerinden daha yüksek bulunması, NT-proBNP'nin kalp yetmezliğini erken dönemde tespit etmede daha hassas bir belirteç olduğunu göstermektedir Introduction: Heart failure due to hemosiderosis is frequent in beta-thalassemia major (?-TM) patients. Magnetic resonance imaging (MRI) is used in the early detection of heart failure. Amino-terminal pro-brain natriuretic peptide (NT-proBNP) is a very sensitive marker in the diagnosis of heart failure. In this study, we aimed to investigate the efficacy of NT-proBNP levels in thalassemia patients, who are thought to have no cardiac iron deposition according to T2* scoring system (CMRT2*?>?20 msn), in early identification of cardiac failure.Methods: NT-proBNP levels of 31 patients, who have T2*?>?20 ms, and of 25 healthy population were measured by chemoluminescence method.Results: NT-proBNP levels were not different in thalassemic patients [median: 33 (IQR: 28–94) pg/mL] compared to control group [median: 41 (IQR: 28–59) pg/mL]. We found that NT-proBNP level was above cut-off value in six patients.Conclusion: NT-proBNP is a cheaper, reachable, and noninvasive method compared to MRI technique, it can be easily used in monthly controls. Detection of highNT-proBNP levels above cut-off values in patients whose T2* values are normal indicates that measurement of NTpro-BNP is a more sensitive marker in early detection of cardiac failure.

0000-0001-9178-2850; WOS: 000406254200010 PubMed ID: 29033512 Background: Abnormal heart rate recovery (HRR) is predictive of cardiac mortality. Autonomic abnormalities in beta-thalassemia major (TM) patients have been reported in previous studies. However, the importance of low HRR in exercise stress test in TM patients has not yet been ascertained. Therefore, this study will be the first of its kind in the literature. Methods: Exercise stress test was performed on 56 TM patients who were being treated at the Thalassemia Center of our hospital, along with 46 non-TM iron deficiency anemia (IDA) patients as a control group. Values for HHR were recorded at 1, 2, 3, 4 and 5 min, and HRR was calculated by the difference of heart rate at peak exercise and at a specific time interval following the onset of recovery. Results: All HRR values were found to be lower in TM patients compared to those in the IDA group. Exercise capacity [metabolic equivalents (METs)] was also found to be low in these patients (p < 0.001) as well. Total exercise time was significantly lower in the TM group compared to the IDA group (8.40 +/- 1.7 min vs. 11.17 +/- 1.51 min, p < 0.001). Exercise capacity (METs) was also lower in the TM group compared to the IDA group. Mean T2* value was 28.3 +/- 13.7 ms in TM patients on magnetic resonance imaging (MRI). In addition, there are 18 TM patients with T2* value was < 20 ms. Conclusions: This study found that TM was independently associated with low HRR. Such a condition is an indicator of autonomic dysfunction in TM patients, since abnormal HRR is related to impaired autonomic response. In addition, impaired HRR may be a marker of early cardiac involvement in patients, whose T2* value is high on MRI. Modifying HRR with a cardiac rehabilitation program in TM patients with impaired HRR is a field open for further investigation.

0000-0001-9178-2850; 0000-0002-5727-9474 WOS: 000437527600011 Objective: beta-thalassemia major (BTM) is a genetic disorder necessitating frequent blood transfusions, which is characterized by functional and physiological disorders in multiple organs. We aimed to research the prevalence of associated skin, hair, and nail disorders, and their relationship with clinical and laboratory features of the disease in adult BTM patients and healthy control groups. Materials and Methods: We prospectively analyzed the prevalence of associated skin, hair, and nail disorders. We recorded demographic features, laboratory findings skin, hair, and nail disorders in the participants. Results: Atotal of 71 BTM patients (36 males and 35 females) and 50 age and sex matched healthy individuals (36 females and 36 males) were included in the study. 32 (45.1%) of the patients and 3 (4.2%) of the control groups had hair, 26 (36.6%) of the patients and 6 (8.3%) of control groups had nail diseases. The most common skin findings xerosis were determined in 66 (93%), hair findings, telogen effluvium were determined in 16 (22.5%), and nail findings, dystrophic nail were determined 9 (12.7%) in patients. BTM patients had significantly higher fruquent xerosis, scar, pigmentation disorders, nevus, pruritus, infections, nail and hair disorders than control groups. Average age of patients with pruritus and nevus were significantly enhanced. Postinflammatory hyperpigmentation and melanocytic nevus in male patients had more higher than female patients. Telogen effluvium in female patients was more higher than male patients. Ferritin levels of the patients with ephelide, diffuse bronze pigmentation (DBP) and telogen effluim had significantly enhanced. Conclusion: Age, gender, medication history and ferritin level are important factors for the development of dermatological disorders among BTM patients.

58th Annual Meeting and Exposition of the American-Society-of-Hematology (ASH) - DEC 03-06, 2016 - San Diego, CA 0000-0001-9178-2850 WOS: 000394452701139 Introduction: Thrombotic microangiopathies (TMAs) are a group of life-threatening disorders characterized with Coombs negative microangiopathic hemolytic anemia (MAHA), thrombocytopenia and variable degrees of tissue injury associated with microvascular thrombosis. According to underlying pathophysiological mechanisms and treatment approaches TMAs can be sub-classified as thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), atypical HUS (aHUS) and secondary TMAs. Here we report the retrospective experience of ThREG (Turkish Hematology Research and Education Group) in patients who were diagnosed with TMA and received therapeutic plasma exchange (TPE). Methods: All consecutive patients who were diagnosed with TMA in last 5 years and treated with TPE by participating centers were included. In order to have a more reliable classification of TMAs, patients without available ADAMTS13 activity at diagnosis were excluded. In all patients serum samples were send for ADAMTS13 activity and anti-ADAMTS13 antibody assays before of TPE. Patients without a secondary etiology of TMA, who had an ADAMTS13 activity < 10% or 10-20% with anti-ADAMTS13 antibodies were diagnosed as TTP. Cases with a known clinical condition associated with TMA were classified as secondary TMA. Patients presenting with renal failure (creatinin > 1.5 mg/dl) who did not belong to TTP or secondary TMA groups, were regarded as HUS/aHUS. All other patients were defined as unclassified TMA. Responses to TPE were evaluated using established criteria defined for TTP. All the data were extracted from medical records of patients. Results: A total of 159 patients (104 females and 54 males) from 19 centers were included. The median age of the study cohort was 36 (14-84). 71 (44.7%), 35 (22%), 27 (17%) and 26 (16.3%) patients were diagnosed as TTP, HUS/aHUS, secondary TMA and unclassified TMA, respectively (Figure-1). 10 of the patients in HUS/aHUS had a diarrhea prodrome. At presentation 77 (48.4%), 100 (62.9%) and 80 (50.3%) patients had fever, neurological abnormalities and renal failure, respectively. 32 (20.1%) patients had classic pentad of TMAs, namely MAHA, thrombocytopenia, renal failure, neurological abnormalities and fever. Patients received median 17 (1-75) plasma volume exchanges for 14 (1-74) days. 85 (53.5%) cases received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 143 patients. 91 patients (63.6%) responded to TPE, while 51 (36.4%) patients had refractory disease. 20 (14%) refractory patients died during follow-up. 32 TPE-refractory patients who were still alive received following treatments: eculizumab (n: 10), increased TPE intensity alone (n: 7), increased TPE intensity plus steroids (n: 9), rituximab (n: 3) and treatment of underlying malignancy (n: 3). Discussion: Most patients presenting with TMAs receive TPE as first line treatment. But distinguishing different types of TMAs is of utmost importance, because treatment should be directed according to underlying pathophysiology. Reliable classification of TMAs in daily practice is problematic and sometimes impossible because of the following reasons: 1. Evidence of secondary TMA may not be evident at diagnosis. 2. Evaluation of mutations of complement system regulators/activators for diagnosis of aHUS and stool culture for STEC-associated HUS are mostly unavailable to community practice. As we were unable to do specific stool cultures, 10 patients with diarrhea prodrome were not regarded as HUS instead put into HUS/aHUS group. Conclusion: As the treatment of various subtypes of TMAs is quite different, we need more powerful and easily accessible tools for differentiation. Until that time, patient presenting with renal failure, no known secondary etiology, and ADAMTS13 activity > 10% should be regarded as aHUS especially if they are unresponsive to first line TPE. In order to plan future treatment strategy, rigorous evaluation for secondary causes of TMAs and evaluation of gene mutations of complement system should follow. Amer Soc Hematol

Amaç: Kronik böbrek hastalığı (KBH) ilişkili malnutrisyon ile pankreatik ekzokrin disfonksiyon arasındaki ilişki henüz net olarak açıklanamamıştır. Böbrek hastalıklarında pankreatik ekzokrin 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., 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.

Tirotoksik periodic paralizi (TPP) çoğunlukla asyalı genç erkeklerde görülen, ani başlayan, hipokalemi ve paralizi ile karakterize bir hastalıktır. Altta yatan hipertiroidizm sıklıkla belirgin olmayıp erken tanı koymak zordur. Aşırı karbonhidrat alımı, aşırı egzersiz, steroid kullanımı ve stress gibi etkenler TPP atağını tetikleyebilir. Biz, bu olguda balık sokması (weever fish) nedeni ile intravenöz deksametazon uygulandıktan sonra akut paralizi gelişen genç erkek hastayı sunduk. Hastanın serum potasyum düzeyi (2.3 mmol/l) ve fosfor düzeyi (1.8 mmol/l) düşük , keratin kinaz düzeyi (609 U/l) yüksekti. Hastada subklinik hipertiroidi saptandı. Renal (24 saat idrar potasyum düzeyi 19.9 mmol/l) ve gastrointestinal potasyum kaybı yoktu. Hastaya tirotoksik hipokalemik periyodik paralizi tanısı kondu. Tedavide akut atakları önlemek üzere nonselektif beta bloker başlandı. Olgumuzu; subklinik hipertiroidi varlığında bile steroid ile TPP atağının tetiklenebileceğine dair farkındalık sağlamak üzere sunmak istedik., Thyrotoxic periodic paralysis (TPP), the most commonly seen disorder among Asian men, is characterized by abrupt onset of hypokalemia and paralysis. The underlying hyperthyroidism is often subtle, which causes difficulty in early diagnosis. Factors such as high-carbohydrate eating habit, excessive exercise, use of steroid, and stress can precipitate a TPP attack. We, hereby, present a young Turkish man who developed acute paralysis after receiving intravenously applied dexamethasone for weever fish poisoning. His serum potassium level was 2.3 mmol/l. Moreover, he had subclinical hyperthyroidism and elevated serum creatinephosphokinase (CPK: 609 U/L) and hypophosphatemia (p:1.8 mg/dl). He had neither a loss of renal (24 h urinary potassium: 19.9 mmol) nor gastrointestinal potassium. His muscle strength and serum potassium were fully recovered after a small amount of potassium replacement. The patient was diagnosed as Steroid induced TPP in subclinical hyperthyroidism and was treated with nonselective beta blockers to prevent the possible attacks. In conclusion, we deduced that Steroid induced TPP may develop even in the presence of subclinical hyperthyroidism. We want to share our experience on this issue to raise awareness

Acute myeloid leukemia (AML) affects middle-aged adults. When the disease involves soft tissue, it is called granulocytic sarcoma (GS). GS can be present in the gastrointestinal tract but involvement of the appendix is uncommon. Furthermore, infiltration of the appendix by leukemic cells is also a rare manifestation of leukemia relapse (1). Here, we report a 50-year-old man with AML who was presented with acute appendicitis and treated successfully by surgery.Keywords: Acute Myeloid Leukemia, Appendisitis, Leukemic Appendisitis, Granulocytic SarcomaÖzetAkut myeloid lösemi orta yaş grubunu etkileyen bir lösemi alt tipidir. Hastalık yumuşak dokuyu tuttuğunda granülositik sarkom olarak adlandırılır. Granülositik sarkom gastrointestinal sistemi tutabilir ancak appendiks tutulumu nadirdir. Buna ek olarak appendiksin lösemik hücrelerle infiltrasyonu şeklinde myeloid lösemi relapsı çok daha nadir bir prezentasyondur. Biz de akut appendisit ile AML relapsı oluşan ve cerrahi ile tedavi edilen 50 yaşındaki erkek olgumuzu sunduk., Acute myeloid leukemia (AML) affects middle-aged adults. When the disease involves soft tissue, it is called granulocytic sarcoma (GS). GS can be present in the gastrointestinal tract but involvement of the appendix is uncommon. Furthermore, infiltration of the appendix by leukemic cells is also a rare manifestation of leukemia relapse (1). Here, we report a 50-yearold man with AML who was presented with acute appendicitis and treated successfully by surgery

Helicobacter pylori (H pylori) populasyonun%50'sinden fazla görülen ve gastrik mukozaya yerleşenspiral şekilli, flajelli, mikroaerofilik, gram (-) bir basildir.Dünya genelinde en yükseği gelişmekte olan ülkelerdebildirilen değişken bir görülme sıklığına sahiptir. Riskfaktörleri ile ilgili çalışmalar özellikle sosyoekonomikfaktörler üzerinde durmaktadır. İnsanlarda gastrit ve ülserile ilişkisi net olarak kanıtlanmıştır. Enfeksiyon çocuklukçağında sıklıkla oral yolla bulaşmaktadır. Üre nefes testi,dışkı antijen testi, antikor tayini, endoskopi, histolojikinceleme, üreaz testi ve kültür tanıda kullanılanyöntemlerdendir. Antibiyoterapi ve antiasitler tek başınayeterli olmadığından birlikte kullanımları tercihedilmektedir. N-asetilsistein gibi mukolitik bir ajan ile Hpylori tabakasının ortadan kaldırılması da tedaviöncesinde etkili olabilmektedir. Lactobacillus,Saccharomyces, Bifidobacterium ve Bifidobacteriumclausii gibi probiyotik suşların eklenmesi de diğer birtedavi yaklaşımıdır. İlk tercih tedaviler yetersizkaldığında farklı antibiyotikleri içeren ikinci adımtedavilere gerek duyulabilmektedir, Helicobacter pylori (H pylori) is a spiralshaped,flagellated, micro- aerophilic gram-negativebacillus that colonizes the gastric mucosa of more than50% of the human population. There are differentfindings for the prevalence of H pylori across the worldwith the highest prevalence in developing countries. Mostof the reports on risk factors focused on socioeconomicindicators. Its relationship with gastritis and peptic ulcerin humans was proven. The infection is transmittedwithin the family in childhood, likely by oraltransmission. Urea breath test, stool antigen test, antibodydetection, endoscopy, histology, urease test, and cultureare used for the diagnosis. Antibiotics and antiacidics arenot sufficient alone, therefore combination treatment ispreferred. Pretreatment with N-acetylcysteine as amucolytic agent to destroy the biofilm of H pylori iseffective. The addition of probiotics such asLactobacillus spp., Saccharomyces spp., Bifidobacteriumspp., and Bifidobacterium clausii as an adjunctive agentis another approach. If the first-line therapy fails, thesecond-line options should include different antibiotics.

ÖzetDiyabetes mellitus tüm dünyada giderek artan sıklıkta görülmektedir ve Son dönem böbrek yetmezliği (SDBY)’nin en sık sebebidir. Kronik böbrek yetmezliği durumunda karbonhidrat ve insülin metabolizmasında oluşan değişiklikler ve diyabet için kullanılan ilaçların renal klirensinin değişmesi kan şeker regülasyonun da zorluklara neden olduğundan dolayı SDBY hastalarında diyabet tedavisi özellik arz etmektedir. Bu yazıda 80 yaşında son dönem böbrek yetmezliği olan bir tip 2 diyabetes mellitus vakasında meglitinid kullanımına bağlı gelişen nadir bir durum olan hipoglisemiyi irdelemek amacı ile sunulmuştur., Prevalence of the diabetes mellitus (DM) is increasing in whole world and it is the most common reason of end stage renal disease. At chronic renal failure condition alteration of carbonhydrate and insulin metabolism and alteration of the renal clearance of the drugs using for diabetes cause difficulties of blood glucose regulation for this reason diabetes treatment is very important for end stage kidney disease patients. In this case presented for examine rare condition hypoglycemia linked using of meglitinide at patient who at 80 years old have type 2 DM and end stage kidney disease.

In developed countries, more than 30% of patients admitted to hospitals are diagnosed anemia. Yet, factors such as patient incompatibility, improper treatment, neglected research of the etiological factors make anemia a public health issue. This study aims to find out the etiology, related risk factors, treatment modalities and evaluation of follow-up in the patients with anemia. Seven hundred and twenty patients admitted to the adult hematology out-patient clinic between January 2011 and January 2012 were evaluated prospectively. Patients with active malignancy, chronic disease anemia, thalassemia major or intermedia and the patients, who did not complete diagnostic tests, were excluded. The remaining 561 patients were included in the study. SPSS for Windows 13.0 was used for the statistical analysis. Mean age was 36.1±17.0 years. Among the patients females were 90.7% and males 9.3%. According to the etiology: iron deficiency anemia; 43.8%, vitamin B12 deficiency; 7.6%, folic acid deficiency; 0.4%, iron deficiency and vitamin B12 deficiency together; 43.8%, iron deficiency and folic acid deficiency together; 1.8%, and folic acid deficiency and vitamin B12 deficiency anemia were 2.6%. Gender did not differ in the patients with vitamin B12 deficiency and folic acid deficiency (p=0.067 and p=0.597). Iron deficiency anemia and dimorphic anemia were more common in women than in men (p, Gelişmiş ülkelerde hastanelere başvuran hastaların %30’undan fazlasında anemi saptanmaktadır. Fakat hasta uyumsuzluğu, uygunsuz tedaviler, etiyolojik nedene yönelik araştırmaların yapılmaması gibi etmenler anemiyi tedavisi zor bir halk sağlığı sorunu haline getirmektedir. Bu çalışmada anemi saptanan hastalarda anemi etiyolojisi, etken risk faktörlerinin belirlenmesi, uygulanan tedaviler ve takibin değerlendirilmesi amaçlanmıştır. Hastanemiz erişkin hematoloji polikliniğine Ocak 2011 - Ocak 2012 tarihleri arasında başvuran ve anemi saptanan 720 hasta prospektif olarak incelendi. Aktif malignite, kronik hastalık anemisi, talasemi major veya intermedia tanısı olan hastalar ve tanısal testleri eksik olanlar çalışma dışı bırakıldıktan sonra kalan 561 olgu çalışmaya alındı. Bulguların değerlendirilmesinde istatistik analizler için SPSS for Windows 13.0 programı kullanıldı. Hastaların %90.7’si kadın, %9.3’ü erkekti. Yaş ortalaması 36.1±17.0 yıldı. Hastalık etiyolojisine göre: demir eksikliği %43.8, B12 vitamini eksikliği %7.6, folik asit eksikliği anemisi %0.4 saptandı. Hastaların % 43.8’inde demir ve B12 vitamini, %1.8’inde demir ve folik asit, %2.6’sında ise B12 vitamini ve folik asit eksikliği anemisi birlikteydi. Hastalarda B12 vitamin eksikliği (p=0.067) ve folik asit eksikliği anemilerinde (p=0.597) cinsiyet dağılımında anlamlı fark yoktu. Demir eksikliği anemisi (p

0000-0001-9178-2850 WOS: 000388367600001 PubMed ID: 27570707 PNH Education and Study Group ( PESG) have been established in December 2013 as a non-profit, independent, medical organization www.pesg.org. Paroxysmal Nocturnal Hemoglobinuria ( PNH) is a multi-systemic disease that should be treated with a multidisciplinary approach. Patients may apply to the clinics other than the hematology due to variability and diversity of clinical findings which lower the rate of diagnosis due to low awareness about PNH. PNH might be overlooked and diagnosis might be delayed. Regarding these, PESG was established with the collaboration of Immunology, Cardiology, Thorax Diseases ( Pulmonology), Neurology, Gastroenterology, General Surgery and Urology specialists in addition to hematologists dealing with PNH. The PESG study group aims to increase the awareness about PNH, including training activities about PNH, strengthening the relations between clinics and planning of clinical studies as a goal. It is the first professional organization focusing on PNH, in Turkey. In this guideline, we want to facilitate the diagnosis attributes of physicians from all specializations that deal with PNH and its systemic complications. One can perceive this as a tailor made guideline of international guidelines but not a compilation.

Hodgkin’s Lymphoma is a tumor that comprises fewer malignant Hodgkin, Reed-Sternberg cells and variants in the tumor tissue (1-2% of all cells) different from the other hematological and solid tumors. Most of the tumor mass is composed of reactive inflamatory cells (T-B lymphocytes, eosinophils, plasmocytes, mastocytes and neutrophils), stromal cells and connective tissue surrounding the tumor cells. Among them, eosinophils frequently infiltrate Hodgkin’s Lymphoma tissues and the peripheral blood eosinophilia is also a well recognised feature (15%) of this disease. However the prognostic importance of this is still controversial. In the literature some of the studies have reported that eosinophilia has no prognostic significance in Hodgkin’s Lymphoma. However some of them claimed that selective eosinophilia without generalised leucocytosis provided clear survival advantage especially in patients with disseminated disease. Here we report a case diagnosed as Hodgkin’s Lymphoma, mixed cellularity type, presenting with a large mass localized in the inguinal region accompanying with tissue and peripheral blood eosinophilia with generalised leucocytosis who had a good survival, Hodgkin Lenfoma diğer hematolojik ve solid tümörlerden farklı olarak az sayıda (tüm hücrelerin %1-2’si) malign hücre yani Hodgkin, Reed-Sternberg hücreleri ve varyantlarını içeren bir tümördür. Tümör kitlesinin çoğunluğu, tümör hücrelerini saran reaktif inflamatuar hücreler (T-B lenfositler, eozinofiller, plazmositler, mastositler ve nötrofiller), stromal hücreler ve konnektif dokudan oluşur. Bu hücrelerden eozinofiller en sık olarak Hodgkin Lenfoma dokusunu infiltre eden hücrelerdendir. Hastanın Periferik kanında eozinofili ise bu hastalığın sık görülen (%15) özelliklerinden biridir. Ancak bu bulgunun prognostik önemi hala tartışmalıdır. Literatürde bazı çalışmalar Hodgkin Lenfoma’da eozinofilinin prognostik bir öneminin olmadığını bildirmişler. Ancak diğer bazı çalışmalarda ise özellikle yaygın hastalığı olan hastalarda; genel lökositoz olmaksızın görülen selektif eozinofilinin sağkalıma belirgin olarak olumlu etki yaptığı bildirilmektedir. Bu nedenle burada Mikst selüler tip Hodgkin Lenfoması inguinal bölgesinde dev bir kitle halinde ortaya çıkan, genel lökositozu olmaksızın belirgin periferal kan eozinofilisi ve doku eozinofilisi bulunan ve iyi bir sağkalıma sahip olan bir hastamızı sunmaya değer bulduk.