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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

Introduction The commonest indication for an Autologous Stem Cell Transplantation (ASCT) is still Multiple Myeloma. A successful mobilization of hematopoietic stem cells (HSC) is a sine qua non of ASCT. The introduction of Plerixafor, which is a partial agonist of the alfa-chemokine receptor CXCR4 has added an important value and impact on mobilization. Plerixafor is successfully integrated into both growth factor-only and cyclophosphamide and growth factor mobilization strategies with significantly reducing the mobilization failure rate in myeloma patients. In addition, plerixafor + G-CSF has also been shown to successfully mobilize the majority of patients who previously failed to mobilize with either growth factor alone or in combination with chemotherapy. Even a Just-in-Time algorithm which induces plerixafor in patients who lacks a certain threshold of CD34 positive HSCs on the day of mobilization led to a cost-effective and successful mobilization with highly restricted rates of mobilization failure. In this study we tried to demonstrate the efficacy and safety of a novel generic Plerixafor (Pleksor - Gen Ilac) and to compare it with original one (Mozobil - Sanofi) in a retrospective manner. Method Patients who were transplanted in two centers who adopted the same mobilization standard operating procedures (SOP) were included in the study. An age and sex matched cohort of patients who received Mozobil (from 2020-2022 - Group A) were compared with the ones who received Pleksor (2021-2022 Group B) as a Just-in-Time conjunct to GCSF alone or chemo mobilization. Poor mobilization was defined as a final yield of 2 million CD34 positive HSCs per kg. Our aim was to collect enough stem cells for at least two ASCTs, thus our current SOP's indicated a minimum CD34 positive HSC threshold of at least 4 million per kg and an ideal HSC threshold of 6 million per kg. Results A total of 28 patients were included and they were equally distributed among Group A (n=14) and B (n=14). Median age of the patients at the time of mobilization were as follows, 60 (35-72) in Group A and 61 (38-70) in Group B. 14 patients who received Pleksor achieved a median yield of 8.40 million CD34 positive HSCs per kg (4.8-21) and the patients who received Mozobil have ended with a yield of 6.7 million CD34 positive HSCs per kg (4.5-13) (p=0.210). None of the patients in both groups were named to be a poor mobilizer according to the threshold of 2 million CD34 positive HSCs per kg but 3 of the patients in Group A and 2 of the patients in Group B ended with a yield of 6 million CD34 positive cells which was below to the ideal threshold for two transplants. Regarding lenalidomide exposure before mobilization, history of radiotherapy, line of the therapies received before mobilization, number of leukapheresis and the mobilization policy (chemo vs gcsf alone) there were no statistically significant difference between two groups (p=0.120, 0.702, 0.842, 0.769 and 0.420 respectively). The median neutrophil engraftment time in days were as follows for Group A and B, 11(10-14) vs 11 (10-16), p=0.541 and the median platelet engraftment time in days were 17 (10-30) in Group A and 16 (10-28) in Group B with a p value of 0.571. In none of the cases any specific side effects were noted which could be attributable to Pleksor or Mozobil. Conclusion Our study demonstrated a comparable efficacy of a generic form of Plerixafor when compared with the originator. This would lead to a decrease in the cost of total process of mobilization with a similar efficacy and toxicity profile. We are now planning to initiate a prospective trial to validate these results in a larger patient population. Up to our knowledge this is the first study comparing the efficacy of a generic Plerixafor in a sole myeloma patient cohort.

Per WHO 2016 and 2022 (5th ed.) myeloproliferative disease guidelines, Chronic Myeloid Leukemia (CML) is classified under two major groups according to the presence of bcr-abl translocation; these groups require different treatment approaches and show clinical presentation heterogeneity. Treatment agents such as tyrosine kinase inhibitors (Imatinib, Dasatinib, Nilotinib, Bosutinib, Ponatinib, Radotinib), Omacetaxine and Asciminib have been used in the treatment of Bcr abl positive CML according to the patient's clinic and mutation status. According to the IRIS study, a study evaluating CML patients treatment response to imatinib, the major molecular response was 33.3% at 3 months, the major molecular response was 48% at 6 months, and the major molecular response was 62.1% at 12 months; furthermore, the rate of achieving a complete molecular response at 12 months was 94.9% (4). In patients who was treated with imatinib as first line therapy, the rate of transformation to accelerated or blastic phase at 18 months was 0.9% in the MMR group and 9.9% in the non-MMR group. In "conventional" CML patients, high leukocyte counts may be accompanied by thrombocytosis; though presentation with only thrombocytosis without leukocytosis is hardly described so far. In this poster presentation, we introduced 7 cases who initially presented with isolated thrombocytosis and then diagnosed with Ph(+) CML. This study was conducted in three adult hematology centers from Antalya, Izmir and Istanbul. 400 patients followed in these centers were reviewed retrospectively; seven patients presented with isolated thrombocytosis were identified. Demographic characteristics, diagnostic findings, and risk scores of these patients were evaluated (Tablo 1). Eln 2013 response criteria were used for evaluation of response for 3rd, 6th, 12th. monthly responses (Figure 1). Here we present 7 CML patients without leukocytosis who were diagnosed with marked thrombocytosis. The patients had similar symptoms and physical examination with no obvious splenomegaly or thrombosis. All of the patients had minimal basophilia and normal peripheral smear findings. All patients responded well to imatinib therapy. During follow up patients who maintained their MMR achieve had a better clinical course and prognosis compared to other CML patients.

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.

Introduction: There is a paucity of data regarding the best practice to institute iv Daratumumab (D) in morbid obese patients regarding the infusion rate and duration, optimal dosing, and ideal way to cope with the infusion related reactions (IRR). We wanted to present the fi rst split dose D infusion experience in a morbid obese MM case whose D was interrupted because of a grade 3 IRR.

Introduction: Treatment of the elderly patients who have varying degrees of renal failure at the time of presentation, both due to the underlying plasma cell dyscrasia or the other comorbidities is a true challenge regarding the management of toxicities while trying to achieve the best durable responses. Daratumumab Lenalidomide and Dexamethasone (DRd) induction in transplant ineligible patient population is approved among the appealing results of MAIA trial. Lenalidomide is an immunomodulatory agent which is eliminated through kidneys and should potentially threat kidney function. But recently, PrE1003 trial showed that even patients with severe renal insuffi ciency should tolerate higher doses of the drug label.

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.

Amaç: Paroksismal nokturnal hemoglobinüri (PNH), kronik intravasküler hemoliz bulguları,kemik iliği yetersizliği ve trombozla kendini gösterebilen edinsel, klonal bir hematopoietikkök hücre hastalığıdır. Klinik bulguların çok değişken olması, tanıda gecikmelere vehastalığın morbi-mortalitesinde artışa yol açabilmektedir. Tanıda altın standart yöntem, temelmoleküler defektin, yani glukozilfosfotidilinozitol (GPİ) çıpası yardımıyla hücre membranınabağlanan proteinlerdeki eksikliğin akım sitometrik yöntemle ortaya konulmasıdır.Tedavisinde ise tek küratif yöntem allojeneik kök hücre naklidir. Ancak günümüzde PNHtedavisinde, hemoliz, transfüzyon bağımlılığı, organ hasarı ve semptomları azalttığı gösterilenve bir terminal kompleman inhibitörü olarak görev yapan eculizumab da yaygın olarakkullanılmaktadır. Bu çok merkezli çalışmada amacımız, ülkemizdeki PNH hastaların kliniközelliklerinin ve tedavi yönetimlerinin değerlendirilmesidir.Gereç ve yöntem: Çalışmamıza yirmi farklı merkezin hematoloji kliniğinde PNH tanısı alantoplam 60 olgu dahil edilmiştir. Tüm olguların retrospektif olarak dosyaları taranmış,hastaların demografik özellikleri, laboratuvar bulguları, gelişen komplikasyonlar, akımsitometri bilgileri, aldıkları tedaviler ve ölüm sebepleri kaydedilmiştir.Bulgular: Çalışmamıza alınan 60 hastanın ortanca yaşları 33 (17-77) idi. Kırkaltı hasta klasikPNH ve 14 hasta sekonder PNH (13 hasta aplastik anemi+PNH, 1 hasta myelodisplastiksendrom+PNH) idi. Hastaların temel demografik özellikleri, hematolojik parametreleri,semptom ve bulguları Tablo1’de özetlenmiştir. Halsizlik ve karın ağrısı en sık başvuruyakınmaları olup, organomegali sadece 7 hastada saptanmıştı. Akım sitometrik analizdeortanca granülosit ve monosit klonları klasik PNH olgularında sırasıyla %75 (23.1-99.5) ve%77 (14.2-99) ve sekonder PNH olgularında %70.5 (13.8-95) ve %61 (14-95.5) olarak izlendi. Tromboembolik olaylar 60 hastanın 18 inde (%30) gözlenmişti. Klasik ve sekonderPNH olguları arasında tromboembolik olay gelişimi açısından istatistiksel bir farkgözlenmemişti (sırasıyla %17.3 ve %14.2, p:0.57). Hastaların, 2009 yılında eculizumabülkemizde ulaşılabilir olana dek immünsupresif ilaçlarla tedavi gördükleri, bu tarihten sonraise olguların %67.4’ünde (31/46) eculizumab tedavisine geçildiği izlendi. Ortalama yaşamsüresi 42 ay (7-183) olarak belirlendi. 60 hastadan 4’ü (%6.6) infeksiyon, 2’si fungalpnömoni, 1’i sepsis ve diğer 1 hasta ise CMV enfeksiyonu nedenli kaybedildi.Tartışma: Çalışmamızda, nadir bir hematopoietik kök hücre hastalığı olan PNH’nin görülmeyaşının literatürle benzerlik gösterdiğini görülmüştür. Trombotik komplikasyon sıklığı %30olarak belirlenmiş olup, sıklıkla ve beklendiği üzere venöz tromboz şeklinde geliştiğigörülmüştür. Literatürden farklı olarak tromboz nedenli ölen hasta görülmemiş, en sık ölümsebebi enfeksiyon olarak belirlenmiştir.Çalışmamız, PNH hastalarının değerlendirildiği ve ülkemizde bugüne kadar yapılmışen kapsamlı çalışmadır. Bu ve benzer çalışmaların, nadir görülen PNH gibi hastalıklarda,ileriye yönelik olarak hastalık yönetimini de etkileyebilecek çok değerli bilgiler verebileceğinidüşünüyoruz.Anahtar kelimeler: Eculizumab, Paroksismal nokturnal hemoglobinüri, Tromboz&nbsp

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.

Hematopoietic stem cells (HSCs) ensure the lifelong production of blood cells throughout a lifetime. Latexin (Lxn) is thought to have a tumor suppressor role and endogenously down-regulate the number of HSCs via increased apoptosis. Therewithal, Raptor, and Rictor are components of the mammalian target of rapamycin complex-1, and 2 (mTORC), which are the regulatory structures for cell growth. However, Lxn, Raptor, and Rictor-associated molecular mechanisms underlying leukemia-induced HSCs proliferation are largely unknown. Nowadays, chronic lymphocytic leukemia (CLL) remains the most common leukemia type in adults. Therefore, we investigated the serum levels of Lxn, Raptor, and Rictor in CLL patients. We randomized 40 patients with newly diagnosed, untreated CLL. Serum levels of Lxn, Raptor, and Rictor were examined using ELISA assay. The results showed that serum Lxn levels reduced in patients with CLL. Moreover, the Rictor level increased in association with the up-regulation of leukocytosis. Although there was a tendency for an increase of the Raptor levels, the differences did not reach statistical significance. The up-regulated Raptor and Rictor levels in CLL suggested that it was associated with cancer pathogenesis. However, decreased Lxn levels raised the question of whether the disease is secondary to epigenetic features or if it is caused by pathology related to Lxn. The negative correlation between Lxn and Raptor/Rictor levels can provide new methods for the treatment of CLL, which are likely to increase the quality of life and improve the prognosis of the disease. In conclusion, further clinical studies are needed to elucidate the role of Lxn and Raptor/Rictor with the newly defined molecular properties in hematological malignancies and the clinical implications of their use. [Med-Science 2021; 10(2.000): 511-5]

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.

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 (100.000/mm3), kısmi yanıt (30.000-100.000/mm3 veya tedaviden sonra trombosit sayısının bir kat artmış olması) ve yanıtsızlık (, NA

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.

WOS: 000418649300017 PubMed ID: 29267448 Deferasirox is an iron chelator agent used in the treatment of diseases with iron overload, such as thalassemia and myelodysplastic syndrome. Although the majority of adverse reactions of deferasirox involve gastrointestinal symptoms and increase in serum creatinine and transaminases, skin rashes, such as maculopapular and urticarial eruptions, have also been reported. This study reports a case of myelodysplastic syndrome with urticarial vasculitis due to deferasirox therapy. Drug eruption was been confirmed by means of a challenge test, together with histopathological and clinical findings. To the best of our knowledge, we report the first case of deferasirox-induced urticarial vasculitis. Physicians should be aware of the possibility of urticarial vasculitis on deferasirox therapy and the fact that the discontinuation of the drug generally results in improvement.

WOS: 000407326300014 PubMed ID: 28824242 Iron overload is one of the major prognostic factor in thallassemia patients. We aimed to evaluate iron accumulation in the heart and liver by MRI in thalassemia major, thalassemia intermedia, and S- thalassemia patients and to examine its association with ferritin and hepcidin levels. Serum ferritin and hepcidin levels were recorded. Iron overload (IOL) in the heart and liver parenchyma was determined based on the standardized T2* and R2 values measured by MRI. The results were evaluated considering the tissue iron overload, serum ferritin and hepcidin levels. Comparing the 109 patients with the 30 healthy controls revealed the mean age: 24.4 +/- 11 versus 31.2 +/- 5 years, median levels of serum ferritin: 1693 versus 40 ng/mL, and hepcidin: 1.94 versus 0.355 ng/mL; p < 0.001, respectively. Comparison of age, serum ferritin and hepcidin levels and MRI findings of the patients with or without IOL revealed that, ferritin and T2* were significantly different in the patients with IOL in cardiac tissue (p = 0.004 and p < 0.001), and, age, ferritin and R2 were significantly different in the patients with IOL in liver tissue (p = 0.036, p < 0.001 and p < 0.001). The MRI-based T2* and R2 values were moderately and inversely correlated with serum ferritin (r = -0.37; p < 0.001 and r = -0.46; p < 0.001). No correlations were found between the MRI-based T2*, R2 values and serum hepcidin. A moderate and positive correlation existed between serum ferritin and hepcidin (r = 0.45; p < 0.001). We considered that, enhanced intestinal iron absorption characterized by decreased serum hepcidin levels in the intervals between successive transfusions were resulted in iron accumulation in our patients.

WOS: 000376969600012 PubMed ID: 29900958 In this article, we report a case with febrile neutropenia occurring after implementation of intraocular bevacizumab. A 60-year-old female patient who had received intravitreal bevacizumab applied to hospital for oral lesions accompanying fever and neutropenia. In spite of empiric antibiotherapy, patient's clinical condition did not improve. Her sputum culture yielded Stenotrophomonas maltophilia. Trimethoprim/sulfamethoxazole and levofloxacin were commenced according to antibiogram. In immunologic evaluation, antinuclear antibodies and anti-double stranded deoxyribonucleic acid were positive as compatible with systemic lupus erythematosus. After proper antibiotic treatment, patient's clinical symptoms resolved. Development of severe neutropenia after procedures which are not expected to create systemic adverse effects should bring to mind underlying immunosuppressive diseases such as systemic lupus erythematosus.