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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.

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.

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.