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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.

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.

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.

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.

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

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.