Search

Pralatrexate is a new generation antifolate treatment agent used for the treatment of relapsed or refractory peripheral T-cell lymphomas. This study aims to determine the general characteristics of the patients receiving pralatrexate therapy in Turkey, contributing to the literature on the effectiveness of pralatrexate therapy in peripheral T-cell lymphomas by determining the response levels of such patients to the therapy. The study also attempts to clinically examine the major side effects observed in patients during treatment with pralatrexate.The study included patients with peripheral T-cell lymphoma followed up in the hematology units of several hospitals in Turkey. Overall, 20 patients aged 18 and over were included in the study.The median age at the time of diagnosis was 58.5 years. PTCL-NOS (Peripheral T-cell lymphoma, not otherwise specified) subtype was in 40% of patients, making the PTCL-NOS the most common subtype in the study. In general, most patients were diagnosed with disease at an advanced stage. Pralatrexate therapy was given to the patients at a median treatment line of 3.5. Pralatrexate dose reduction was required in only 3 patients (15%). Response to pralatrexate therapy with partial remission (PR) and above was observed in 11 (55%) of the patients.Pralatrexate seemed to be a promising novel treatment in relapsed refractory PTCL patients. However, patients receiving pralatrexate should be followed up carefully for skin reactions, mucosal side effects, thrombocytopenia and neutropenia.

Langerhans cell histiocytosis, formerly known as histiocytosis X, represents clonal proliferations of the antigen-presenting dendritic cells, which are normally found in many organs. It is a rare disease which tends to affect children and adolescents. In particular, adult-onset type is very rare. Herein, we present a female adult diagnosed with Langerhans cell histiocytosis of the rib without any systemic involvement which was successfully treated with surgery.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare X-linked genetic disorder. On the contrary to its name, it is a multisystemic disease and various symptoms other than hemoglobinuria could be occurred. It could be life threatening especially because of thromboembolic events. In the last decade, a terminal complement inhibition with eculizumab approved with promising results for PNH patients. We conducted this study to evaluate the long term experience of eculizumab therapy from Turkey for the first time. Our cohort included 138 patients with PNH treated with eculizumab between January 2008 and December 2018 at 28 centers in Turkey. Laboratory and clinical findings at the time of diagnosis and after eculizumab therapy were recorded retrospectively. The median age was 39 (range 18-84) years and median granulocyte PNH clone size was 74% (range 3.06-99.84%) at the time of diagnosis. PNH with bone marrow failure syndrome was detected in 49 patients and the rest of 89 patients had classical PNH. Overall 45 patients (32.6%) had a history of any prior thrombotic event before eculizumab therapy and only 2 thrombotic events were reported during the study period. Most common symptoms are fatigue (75.3%), hemoglobinuria (18.1%), abdominal pain (15.2%) and dysphagia (7.9%). Although PNH is commonly related with coombs negativity, we detected coombs positivity in 2.17% of patients. Seven months after the therapy, increased hemoglobin level was seen and remarkably improvement of lactate dehydrogenase level during the treatment was occurred. In addition to previous studies, our real life data support that eculizumab is well tolerated with no serious adverse events and improves the PNH related findings.

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

Objective: This study aimed to retrospectively evaluate the efficacy, safety, and survival outcome of single-agent ibrutinib therapy in chronic lymphocytic leukemia patients. Materials and Methods: A total of 136 patients (mean age +/- standard deviation: 64.6 +/- 10.3 years, 66.9% males) who had received at least one dose of ibrutinib were included in this retrospective multicenter, noninterventional hospital-registry study conducted at 33 centers across Turkey. Data on patient demographics, baseline characteristics, laboratory findings, and leukemia-cell cytogenetics were retrieved. Treatment response, survival outcome including overall survival (OS) and progression-free survival (PFS), and safety data were analyzed. Results: Overall, 36.7% of patients were categorized as Eastern Cooperative Oncology Group (ECOG) class 2-3, while 44.9% were in Rai stage 4. Fluorescence in situ hybridization revealed the presence of del(17p) in 39.8% of the patients. Patients received a median of 2.0 (range: 0-7) lines of pre-ibrutinib therapy. Median duration of therapy was 8.8 months (range: 0.4-58.0 months). The 1-year PFS and OS rates were 82.2% and 84.6%, respectively, while median PFS time was 30.0 (standard error, 95% confidence interval: 5.1, 20.0-40.0) months and median OS time was 37.9 (3.2, 31.5-44.2) months. Treatment response (complete or partial response), PFS time, and OS time were better with 0-2 lines versus 3-7 lines of prior therapy (p, Janssen Pharmaceutica Turkey, This study was supported by Janssen Pharmaceutica Turkey. The authors would like to thank Prof. Sule Oktay, MD, PhD, and Cala Ayhan, MD, from KAPPA Consultancy Training Research Ltd. (stanbul, Turkey) , who provided editorial support.

Gaucher disease is a lipid storage disorder due to deficiency of beta glucocerebrosidase. It's an autosomal recessive disease and as a result of this enzyme deficiency, glucocerebroside accumulates in various types of tissues like liver, brain spleen and bone marrow. We aimed to describe the effects of enzyme replacement therapy in three members of a family with Gaucher disease and to emphasize screening of the family members of the patients with Gaucher disease. Furthermore, late diagnosis and treatment in these patients have a minimal effect on improvement of the quality of life, and early diagnosis and treatment are very important in Gaucher disease. © 2017 National Medical Association

WOS: 000425633300017, PubMed ID: 28707314, Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is a rare subtype of Hodgkin's lymphoma. In this study, we aimed to investigate the clinical features and therapeutic outcomes of patients with NLPHL who were diagnosed at different institutes in Turkey. We retrospectively reviewed the records of the patients diagnosed with NLPHL. Adult patients who were diagnosed after 2005 with histological confirmation were selected for the study. Forty-three patients were included in the study. Median age of patients was 37.5years (18-70) at the time of diagnosis. About 60.5% patients were diagnosed as stage I and II NLPHL, and remaining 39.5% had stage III and IV disease. Median follow-up was 46months. During follow-up, none of the patients died. Seven patients relapsed or progressed after initial therapy at a median of 12months. Five of 7 relapsed/refractory patients (71.4%) were salvaged with chemotherapy only (DHAP, ICE), and the remaining 2 (28.6%) were salvaged with chemoimmunotherapy. All of relapsed/refractory patients were able to achieve complete remission after salvage therapy. Lactate dehydrogenase levels were significantly higher in patients with progressive disease compared with nonprogressive disease. Our study showed an excellent outcome with all patients alive at last contact with a median follow up of 46months despite a wide range of different therapeutic approaches. All relapsed and refractory patients were successfully salvaged despite a low frequency of patients received immunotherapy in conjunction with chemotherapy. Our results suggest that immunotherapy may be reserved for further relapses.

Signet-Ring Plasma Cells A 72-year-old man was referred to the hematology service for management of multiple myeloma. A bone marrow biopsy confirmed 70% involvement of plasma cells, with some cells...

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

Since similar symptoms and findings can be seen in the deficiencies of both iron and zinc, we aimed to evaluate the serum zinc levels of women with iron deficiency anemia (IDA). This study was conducted with women with iron deficiency and a healthy control group. When serum zinc levels were compared, they were found to be lower in the IDA group, which was statistically significant. With the help of these studies, iron and zinc treatment instead of only iron replacement may be considered in cases of iron deficiency.Demir ve çinko eksikliği benzer belirti ve bulgularla giden hastalıklar olması sebebiyle, demir eksikliği anemisi (DEA) olan kadınlarda serum çinko düzeylerinin değerlendirilmesini planladık. Bu çalışma DEA’lı kadınlar ile sağlıklı kontrol olarak alınan kadınlar üzerinde yapıldı. Serum çinko düzeyleri karşılaştırıldığında, DEA grubunda istatistiksel olarak düşük saptandı. Bu çalışmaların yardımıyla sadece demir değil, demir ve çinkonun beraber tedavisi demir eksikliği olan olgularda düşünülebilir.

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

We present a case of Visceral Leishmaniasis (VL) in a patient with gastric cancer treated with chemoradiotherapy. A 69-year-old man with gastric carcinoma had been treated with total gastrectomy, lymphadenectomy and postoperative radiotherapy with chemotherapy. Then 3 years after treatment, due to pancytopenia with progressive trombocytopenia and splenomegaly, bone marrow examination was performed and Leishmania spp. amastigotes were diagnosed. When antibiotherapy was completed, splenomegaly was eliminated and the laboratory results became normal. VL should be kept in mind in the differential diagnosis of patients, even adults, with splenomegaly, trombocytopenia and a history of contact with dogs in endemic countries such as Turkey. © 2018, Springer-Verlag GmbH Austria, part of Springer Nature.

In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty-six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first-line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy (95%) was again cladribine at second-line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28-month median OS was 91.7% in all patients and 25-month median OS 96% for patients who were given cladribine as first-line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population.

Amaç: Kemoterapiye bağlı febril nötropeni (FEN) gelişen hastalarda ölümlerin en önemli nedeni infeksiyonlardır. Bu nedenle nötropenik hastalarda ateş, aksi ispat edilinceye kadar infeksiyon olarak kabul edilip empirik geniş spektrumlu antibiyotik tedavisine hemen başlanılması gerekmektedir. Çalışmamızda kemoterapiye bağlı gelişen 236 FEN atağı; infeksiyon kategorileri, izole edilen patojen mikroorganizmalar, mortalite oranları ve uygulanan antibiyotik tedavileri açısından retrospektif olarak değerlendirildi. Merkezimizdeki infeksiyon etkenlerinin ve tedavi protokollerinin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Çalışmaya, İnönü Üniversitesi Tıp Fakültesi Turgut Özal Tıp Merkezi, Erişkin Hematoloji Kliniği’nde 2002 ile 2010 tarihleri arasında takip ve tedavisi yapılan 87 akut myeloid lösemi (AML) hastası alındı. 236 FEN atağı retrospektif olarak değerlendirildi. FEN kriterlerini karşılayan hastaların ataklarında infeksiyonlar mikrobiyolojik olarak tanımlanmış infeksiyon (MTİ), klinik olarak tanımlanmış infeksiyon (KTİ) ve nedeni bilinmeyen ateş (FUO) olarak kategorize edildi. Hastaların fizik muayene ve kültür sonuçları dosya kayıt sistemi ve mikrobiyoloji kayıtlarından elde edildi. Bulgular: Hastaların 53’ü erkek, 34’ü kadındı. Yaş ortalaması 52.44 yıl olarak bulundu. 236 FEN atağının %30.9’u MTİ, %40.3’ü KTİ ve %28.8’i FUO olarak kategorize edildi. Ataklarının %19.9’unda kan kültüründe etken patojen mikroorganizma izole edilirken, %80,1’inde izole edilmediği saptandı. Kan kültürlerinden izole edilen mikrorganizmaların %51.1’ini gram-pozitif bakteriler, %40.4’ünü gram-negatif bakteriler ve %8.5’ini ise fungal etkenlerin oluşturduğu saptandı. Klinik olarak dökümante edilen 95 ataktan %47.4’ünde pnömoni saptandı. Tüm ataklarlarda ortalama nötropeni süresi 13.33, MDİ’de 16.69, KTİ’de 13.09 ve FUO’da ise 10.04 gün olarak bulundu. Mortalite atak başına %8.5 olarak tespit edildi. Sonuç: Merkezimizde bakteriyemi etkeni olarak gram-pozitif mikroorganizmalar daha sık saptanmıştır. Her merkezin kendi infeksiyon etkenlerini yakından izleyip empirik antibiyotik tedavi politikalarını belirlemesi, FEN sürecinin daha iyi yönetilmesinde olumlu katkı sağlayabilir., Aim: The most important cause of mortality in febrile neutropenic episodes (FNEs) which mature after chemotherapy s infections. Fever in neutropenic patients must be accepted as an infection and broad-spectrum empric antibiotherapy must be started immediately as a standard approach. The infection categories, isolated pathogen microorganisms, mortality ratios and antibiotherapy regimens in 236 FNEs which matured after chemotherapy have been examined retrospectively. Our goal was identification of infectious agents and to determine our treatment protocol. Materials and Methods: Our study includes 87 patients who were treated because of acute myeloid leukemia (AML) at Inonu University Turgut Özal Medicine Center Adult Hematology Clinic between 2002 and 2010. 236 FNEs were examined retrospectively. Infections of patients who met FNE criterias were categorized as microbiologic defined nfection (MDI), clinical defined infection (CDI) and fever of unknown origin (FUO). Physical examination and microbiological culture results were obtained from the records. Results: Fifty-three patients were male and 34 female. The mean age was 52.44 years. Fever was evaluated in 30.9% MDI, 40.3% CDI and 28.8% FUO categories. In FNEs, 19.9% efficient pathogen microorganisms were isolated from blood cultures. 51.1% of pathogens which were isolated from blood cultures were gram-positive, 40.4% were gram-negative bacteries and 8.5% were fungus. Pneumonia was detected 47.4% in 95 CDI episodes. The mean neutropenia duration was 13.33 days in all episodes, 16.69 in MDI, 13.09 in CDI and 10.04 days in FUO. Conclusion: If medical centers follow their infection agents closely and modify their empiric antibiotic treatment policies they can supply a useful additive for a better FEN management process.

61st Annual Meeting and Exposition of the American-Society-of-Hematology (ASH) -- DEC 07-10, 2019 -- Orlando, FL, BERBER, Ilhami/0000-0003-3312-8476, WOS:000577164606194, [No Abstract Available], Amer Soc Hematol

Gaucher's disease (GD) is a rare disease characterized by a β-glucocerebroside accumulation in the reticulo-endothelial system. Patients may refer to the clinic with complaints of bone pain, hepatosplenomegaly, anemia, thrombocytopenia, growth retardation, interstitial pulmonary disease, pulmonary hypertension, and skeletal disorders. Skeletal system involvement is observed commonly in Gaucher patients and a significant cause of morbidity. Our patient was followed for several years as a glass child - osteogenesis imperfecta and he had joint deformities due to skeletal fractures. We wanted to present this case to raise awareness of GD's skeletal involvement and effects of late diagnosis.

Autologous hematopoietic cell transplantation (AHCT) is an established treatment option for adult patients presenting with multiple myeloma (MM), Hodgkin lymphoma (HL) and various subtypes of non-Hodgkin lymphoma (NHL) in upfront and/or relapsed/refractory disease settings. Although there are recently published consensus guidelines addressing critical issues regarding autologous hematopoietic progenitor cell mobilization (HPCM), mobilization strategies of transplant centers show high variability in terms of routine practice. In order to understand the current institutional policies regarding HPCM in Turkey and to obtain the required basic data for preparation of a national positional statement on this issue, Turkish Hematology Research and Education Group (ThREG) conducted a web-based HPCM survey. The survey was designed to include multiple-choice questions regarding institutional practice of HPCM in adults presenting MM, HL, and NHL. The representatives of 27 adult HCT centers participated to the study. Here we report the results of this survey shedding light on the real world experience in Turkey in terms of autologous HPCM mobilization strategies in patients presenting with MM and lymphoma. (C) 2017 Elsevier Ltd. All rights reserved.

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. Figure 1 Distribution of TMAs Figure 1. Distribution of TMAs Disclosures Demirkan: Amgen: Consultancy.

Studies about leukocytapheresis have emerged with the need of search for alternatives to conventional treatment in inflammatory bowel diseases (IBD). Leukocytapheresis is a novel non-pharmacologic approach for active ulcerative colitis (UC) and Crohn's disease (CD), in which leukocytes are mechanically removed from the circulatory system. Patients with active IBD treated with leukocytapheresis using a Cellsorba E column between 2012 and 2015, were enrolled in Turkey. In our experience, the results of leukocytapheresis therapy in 6 patients with CD and 20 patients with active UC were overviewed. Leukocytapheresis (10 sessions for remission induction therapy, 6 sessions for maintenance therapy) was applied to the patients with their concomitant medications. Intensive leukocytapheresis (≥4 leukocytapheresis sessions within the first 2 weeks) was used in 30% patients with active severe UC. The overall clinical remission rate in patients with UC was 80%, and the mucosal healing rate was 65%. Patients were followed for an average of 24 months. It was observed that clinical remission has continued in 65% of patients with UC. Mild relapse was observed in 3 patients with UC during follow up period. In 5 patients with CD significant clinical remission was achieved except only one patient. Surgical needs were disappeared in 3 patients with obstructive type Crohn's disease. Adverse events were seen in only 4.3% of 416 sessions. Any concomitant medications did not increase the incidence of adverse events. Our results indicate that leukocytapheresis is efficacious in improving remission rates with excellent tolerability and safety in patients with IBD. © 2017 Elsevier Ltd

Aims: The aim of this study was to evaluate myocardial performance index (MPI) which reflects the combined systolic and diastolic performance of the ventricles by tissue Doppler imaging (TDI) in patients with polycythemia vera (PV). Method and Materials: Twenty-eight patients with PV (17 men; mean age 60 ± 9 years) and 30 age-matched healthy subjects were prospectively evaluated. The diagnosis of PV was performed according to the World Health Organization (WHO) criteria. Left ventricular (LV) systolic and diastolic functions were assessed by conventional echocardiography and TDI. MPI of both the LV and right ventricles (RV) were measured by TDI method. Results: The LV MPI was significantly higher in PV group than in the controls (0.61 ± 0.16 vs. 0.49 ± 0.05; P = 0.001). Also, the RV MPI was impaired in patients with PV compared to the control subjects (0.51 ± 0.11 vs. 0.43 ± 0.09; P = 0.005). RV late A filling velocity (Am) and RV isovolumetric relaxation time were significantly higher in the PV group compared to healthy subjects (P = 0.03 and 0.05, respectively). In logistic regression models, PV was determined as an independent predictor of impaired MPI (odds ratio: 3.7; CI 95%, 1.2–7.5). In addition, pulmonary arterial pressure was significantly elevated in patients with PV compared to the controls (P = 0.02). Conclusion: This study demonstrated that biventricular MPI is impaired in patients with PV. (Echocardiography 2011;28:948-954)

The combination of melphalan-prednisone-thalidomide (MPT) has been investigated in several clinical studies that differed significantly with regard to patient characteristics and treatment schedules. This prospective trial differs from previous melphalan-prednisone (MP) vs. MPT trials by treatment dosing, duration, routine anticoagulation, and permission for a crossover. Newly diagnosed patients with multiple myeloma (MM) (n=122) aged greater than 55 yr, not eligible for transplantation were randomized to receive 8 cycles of M (9 mg/m(2) /d) and P (60 mg/m(2) /d) for 4d every 6 wk (n=62) or MP and thalidomide (100 mg/d) continuously (n=60). Primary endpoint was treatment response and toxicities following 4 and 8 cycles of therapy. Secondary endpoints were disease-free (DFS) and overall survival (OS). Overall, MPT-treated patients were younger (median 69 yr vs. 72 yr; P=0.016) and had a higher incidence of renal impairment (RI, 19% vs. 7%, respectively; P=0.057). After 4 cycles of treatment (n=115), there were more partial responses or better in the MPT arm than in the MP arm (57.9% vs. 37.5%; P=0.030). However, DFS and OS were not significantly different between the arms after a median of 23 months follow-up (median OS 26.0 vs. 28.0 months, P=0.655; DFS 21.0 vs. 14.0 months, P=0.342, respectively). Crossover to MPT was required in 11 patients, 57% of whom responded to treatment. A higher rate of grade 3-4 infections was observed in the MPT arm compared with the MP arm (22.4% vs. 7.0%; P=0.033). However, none of these infections were associated with febrile neutropenia. Death within the first 3 months was observed more frequently in the MP arm (n=8, 14.0%) than in the MPT arm (n=2, 3.4%; P=0.053). Long-term discontinuation and dose reduction rates were also analyzed (MPT: 15.5% vs. MP: 5.3%; P=0.072). Although patients treated with MPT were relatively younger and had more frequent RI, better responses and less early mortality were observed in all age groups despite more frequent discontinuation.

Objective: The aim of the present case-control study was to determine whether or not the prevalence of gallbladder stones (GBS) was increased in patients with chronic myelocytic leukemia (CML) and to investigate clinical and laboratory characteristics of CML patients with GBS. Subjects and Methods: This study included 56 patients with CML and 55 sex- and age-matched healthy controls. All participants underwent abdominal ultrasonography and the main clinical and laboratory characteristics were recorded. Results: Gallbladder stones were detected in 13 (23.6%) patients with CML and in 3 (5.4%) control individuals (p < 0.05). The mean follow-up period of CML patients after diagnosis was 54.6 months, range 3–120 months. Hemoglobin levels were higher in the control group than in CML patients. However, total bilirubin, unconjugated bilirubin, lactate dehydrogenase levels, leukocyte and thrombocyte counts, frequency of splenomegaly and hepatomegaly were higher in the CML than in the control group (p < 0.05). Other clinical and laboratory values were not significantly different between the groups. CML patients with and without GBS were also compared for clinical and laboratory values. Age and follow-up period of CML patients after diagnosis were higher in the CML patients with GBS (p < 0.05). Conclusions: Higher prevalence of GBS in CML patients than in healthy controls was detected. We suggest that CML may increase the frequency of GBS, apart from other well-known risk factors. This risk is probably related to increased unconjugated bilirubin, which determines hemolysis, older age and long follow-up period of CML patients after diagnosis.

Objective: The aim of the study was to evaluate the role of vitamin B12 in patients with pernicious anemia. Materials and Methods: This study was conducted prospectively at the Turgut Özal Medical Center, Department of Hematology, between April and November 2002. Absolute numbers and ratio of the surface antigens of T and B lymphocyte subgroups, CD4/CD8 ratio were calculated in order to evaluate changes in leukocyte and lymphocyte numbers; natural killer (NK) cell count, serum C3, C4, and levels of immunoglobulins G, A, and M were also measured to evaluate vitamin B12 effect on immunity. Values obtained before treatment with cyanocobalamin were compared with those found during peak reticulocyte count. Results: In vitamin B12-deficient patients, absolute numbers of CD4+ and especially CD8+ lymphocytes were found to be decreased; CD4/CD8 ratio increased, and NK cell activity was depressed. After cyanocobalamin treatment, absolute numbers and percentage of lymphocyte subgroups were elevated. Increased CD4/CD8 ratio and depressed NK cell activity were restored and levels of C3, C4, and immunoglobulins were elevated. Conclusion: These findings suggest that vitamin B12 has important immunomodulatory effects on cellular immunity, and abnormalities in the immune system in pernicious anemia are restored by vitamin B12 replacement therapy.

WOS: 000374614700015, PubMed ID: 26810141, We investigated the course of 54 patients presenting with primary central nervous system lymphoma, who were treated in daily practice. The patients were treated with chemotherapy and/or radiotherapy and/or intrathecal chemotherapy. At a median follow-up period of 23 months (range 1-71), median relapse-free survival (RFS) and overall survival (OS) were not reached. Estimated 2-year RFS and OS rates were 42% and 48%, respectively. Ten relapsed PCNSL patients underwent ASCT. Complete remission rate of these patients was 40%, with 20% treatment-related mortality. Estimated 2-year RFS and OS rates were 37% and 40%, respectively. The prognosis of patients with PCNSL, who received off-study treatment, is still dismal. (c) 2016 Elsevier Ltd. All rights reserved.

Circulating homocysteine is a risk factor of cardiovascular and cerebrovascular events. Hyperhomocysteinemia may be an early indicator for vitamin B12 disorders because cobalamin is a cofactor in the remethylation process of homocysteine. Serum holotranscobalamin (holoTC II) becomes decreased before the development of metabolic dysfunction. In this study, we assessed circulating holoTC II to estimate the diagnosis of vitamin B12 deficiency in the first ischemic cerebrovascular attack. We also compared the efficacy of the measurement of plasma holoTC II with the other standard biochemical and hematological markers used to reach the diagnosis of cobalamin deficiency. Forty-five patients (age 71 years (range 35-90), 16 men/29 women) within the first ischemic cerebrovascular event were included in this prospective study. All the enrolled patients have been administered vitamin B12 1 mg intramuscular injection once a day for 10 days. At the baseline and on the tenth day of treatment, plasma levels of holoTC II and the proper biochemical and hematological markers in diagnosing cobalamin deficiency were measured. After admission, anemia and diminished serum vitamin B12 levels were determined to be only 20% (9/45) and 44% (20/45), respectively; 78% (35/45) of the patients had low serum holoTC II (

Summary Background: Transfusion-associated graft-versus-host disease (TA-GVHD) is extremely rare compared to transplant-associated GVHD. Mortality in TA-GVHD approaches to 100%. We report six cases with TA-GVHD that were seen during 1994‐2004 and discuss the clinical characteristics. Case Reports: Two patients were immunosuppressed, and 4 patients were immunocompetent. All 6 patients were transfused with non-irradiated blood products from relatives before TA-GVHD had occurred. Diagnosis of TA-GVHD in all patients was made based on characteristic clinical manifestations and laboratory findings. All patients died due to TA-GVHD-related complications such as progressive pancytopenia and liver dysfunctions. Conclusion: Our data shows that TA-GVHD is a serious complication of transfusion of blood products

We report four patients with rheumatic disease (RD) and chronic myelogenous leukemia (CML). In two patients with Behcet's disease (BD) and rheumatoid arthritis (RA), CML developed after RD, in two patients with diffuse cutaneous systemic sclerosis and spondyloarthropathy, RD was diagnosed after CML. A variety of interactions have been described between hematological malignancies and RD. Nevertheless, few cases of RD have been documented associated with CML. It is unclear whether the development of CML in patients with RD and RD development after CML occurs by chance alone, is due to the underlying disease, or is facilitated by drugs. Whatever the cause is, it should be kept in mind that CML may develop in the course of RD and RD may be seen in CML patients.

Recent evidence suggests that the activation of platelets and their interaction with circulating cells are important independent risk factors for atherosclerosis. In non-uremic patients with symptomatic peripheral vascular disease, a relationship between serum homocysteine (Hcy) levels and platelet activity had been reported. The purposes of this study were to evaluate of effects of dialysis modality on platelet activity in patients with end-stage renal failure and to investigate the relationship between platelet activity, Hcy, and left ventricular hypertrophy (LVH).In age and sex matched 19 healthy subjects, 20 hemodialysis (HD) patients, and 18 continuous ambulatory peritoneal dialysis (CAPD) patients, the expression of platelet surface receptors CD41, CD61, CD42a, and CD62P were investigated. CD62P expression was statistically significantly increased in HD patients compared with CAPD patients and controls (34.4 +/- 22.5%; 17.3 +/- 19.6%, 12.0 +/- 15.6%, respectively, p0.05), but not in CAPD patients compared with controls. There was a positive correlation between CD62 expression and duration of dialysis in HD patients (r = 0.498, p = 0.026). Mean plasma Hcy levels in dialysis patients were higher than reference levels. However, we could not find any relationship between CD62 expression, Hcy, and LVH in both groups (p0.05).Hemodialysis and peritoneal dialysis (PD) have a different impact on the expression of CD62: peritoneal dialysis seems to have a more favorable effect. It may be possible that the differences in biocompatibility between PD and HD potentially contribute to differences in CD62 expression.

This study investigated changing levels of serum oxidant/antioxidant with chemotherapy and their relation to treatment in 34 Hodgkin's lymphoma patients. The patient population consisted of 19 males and 15 females. Mean age was 30.41 ± 12.08 years. All patients received the adriamycin, bleomycin, vincristine and dexamethasone (ABVD) treatment protocol. Blood samples were taken before treatment, and on days 1 and 7 during treatment for measurement of superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), catalase (CAT), malondialdehyde (MDA), nitric oxide (NO) and enzyme activities. After ABVD treatment, mean free radical levels were increased and antioxidant levels were significantly decreased in the serum. ABVD treatment results in an increase of free radical levels and a decrease of antioxidant levels in the serum of patients with Hodgkin's lymphoma.

Reactive oxygen and nitrogen species have been implicated in the pathogenesis of bleomycin -induced lung fibrosis. The effects of aminoguanidine and erdosteine on the bleomycin-induced lung fibrosis were evaluated in rats. The animals were placed into five groups: Vehicle + vehicle, vehicle + bleomycin (2.5U/kg), bleomycin + aminoguanidine (200mg/kg), bleomycin + erdosteine (10mg/kg), and bleomycin + erdosteine + aminoguanidine. Bleomycin administration resulted in prominent lung fibrosis as measured by lung hydroxyproline content and lung histology, which is completely prevented by erdosteine and aminoguanidine. A strong staining for nitro tyrosine antibody in lung tissue and increased levels of lung NO were found in bleomycin group, that were significantly reduced by aminoguanidine and erdosteine. Aminoguanidine and erdosteine significantly prevented depletion of superoxide dismutase and glutathione peroxidase and elevated myeloperoxidase activities, malondialdehyde level in lung tissue produced by bleomycin. Data presented here indicate that aminoguanidine and erdosteine prevented bleomycin-induced lung fibrosis and that nitric oxide mediated tyrosine nitration of proteins plays a significant role in the pathogenesis of bleomycin-induced lung fibrosis. Also our data suggest that antifibrotic affect of antioxidants may be due to their inhibitory effect on nitric oxide generation in this model. (C) 2004 Elsevier Inc. All rights reserved.

Multiple myeloma (MM) is a neoplastic disorder characterized by monoclonal multiplying of plasma cells. Although radiation, environmental factors, viruses and other factors have been suggested as potential causes of the disease, the aetiopathogenesis of MM is still obscure. This clinical study was designed to investigate the role of reactive oxygen species (ROS) in the aetiopathogenesis of the disease and the possible relationships between treatment and ROS production. For this purpose, erythrocyte superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), and catalase (CAT) activities as well as plasma nitric oxide (NO) and malondialdehyde (MDA) levels were measured in 14 MM patients newly diagnosed at stage III. The relationship between the above-mentioned parameters and vincristine-adriamycin-dexamethasone (VAD) therapy were also investigated in the same patients. All the enzyme activities and the parameters of oxidative stress were found to be significantly reduced after VAD therapy. These findings suggest that ROS may be involved in the aetiopathogenesis of MM. Further investigations with a larger cohort of MM patients are needed to provide definitive data about the role of ROS in MM and the alternative therapeutic approaches to MM.

In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty-six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first-line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy (95%) was again cladribine at second-line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28-month median OS was 91.7% in all patients and 25-month median OS 96% for patients who were given cladribine as first-line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population.

Background. Ineffective erythropoiesis and intramedullary hemolysis are the two important results of pernicious anemia. Hemolysis is one of the risk factors in the formation of gallbladder stones. Patients and methods. The frequency of gallstones was searched in 59 patients with pernicious anemia and in 54 healthy subjects. Results. Nineteen (32.2%) patients in the pernicious anemia group and three (5.5%) patients in the control group were detected to have gallstones. Pernicious anemia patients with and without gallbladder stones were also compared for mean corpuscular volume, hemoglobin, unconjugated bilirubin, lactic dehydrogenase, and vitamin B12 levels but no statistically significant difference was encountered. Conclusion. Apart from other risk factors, pernicious anemia itself increases the frequency of gallbladder stones. Should patients with pernicious anemia develop upper-abdominal pain or other symptoms referable to biliary tract disease, a high index of suspicion for gallstone-related disease should be maintained.

A prospective randomized international study of 143 patients showed no apparent early survival advantage conferred by combining cytarabine, rather than hydroxyurea, with INF as first-line CML therapy. Combinations of alpha-interferon (INF) and chemotherapeutic agents are currently first-line therapy for the majority of patients with chronic myeloid leukemia (CML). The International Oncology Study Group conducted a prospective randomized study comparing INF combined with hydroxyurea or cytarabine. The primary study aim was to compare the survival durations in these patient cohorts. Patients with early chronic phase CML were randomized to receive INF 5 million units (Mu) given five times per week subcutaneously plus hydroxyurea or cytarabine as required to achieve a complete hematologic response and to maintain a WBC count between 2x10(9)/L and 10x10(9)/L and a platelet count between 75x10(9)/L and 100x10(9)/L. Therapy continued as tolerated unless progressive or blast phase disease occurred. At 36 months, the actuarial survival rate was equivalent in both groups: HI group (79 patients) survival was 85% (95% CI, 68-100%), as compared to 95% (95% CI, 79-100%) in the CI group (64 patients). In conclusion if seems that there is no apparent early survival advantage conferred by combining cytarabine, rather than hydroxyurea, with INF as first-line CML therapy.

Iron deficiency anemia is a cause of reactive thrombocytosis. A moderate increase in platelet numbers is common but sometimes counts may exceed 1,000 × 109/l. The mechanisms causing reactive thrombocytosis are unclear. In this study, we evaluated 15 women with iron deficiency anemia and thrombocytosis (platelets >450 × 109/l) and 16 women with iron deficiency anemia with normal platelet counts. Serum samples were taken before oral iron replacement therapy, after 1 and 3 months and at the end of replacement therapy. Thrombopoietin, erythropoietin (EPO), leukemia inhibitory factor, interleukin-6 and interleukin-11 levels were assayed. There was no change in the levels of thrombopoietic cytokines except for EPO. The correlation between high EPO levels and high platelet counts may suggest that EPO increases platelet counts, but the same EPO level changes can also be demonstrated in women with iron deficiency anemia but normal initial platelet counts. The fact that the levels of other cytokines remained unchanged during treatment suggests that either these cytokines have no effect on reactive thrombocytosis or the change in platelet counts in our patients is in a narrow range and is thus not affected by the cytokine levels.

Introduction. Different ferric and ferrous iron preparations can be used as oral iron supplements. Our aim was to compare the effects of oral ferric and ferrous iron therapies in women with iron deficiency anaemia.Methods. The present study included 104 women diagnosed with iron deficiency anaemia after evaluation. In the evaluations performed to detect the aetiology underlying the iron deficiency anaemia, it was found and treated. After the detection of the iron deficiency anaemia aetiology and treatment of the underlying aetiology, the ferric group consisted of 30 patients treated with oral ferric protein succinylate tablets (2 × 40 mg elemental iron/day), and the second group consisted of 34 patients treated with oral ferrous glycine sulphate tablets (2 × 40 mg elemental iron/day) for three months. In all patients, the following laboratory evaluations were performed before beginning treatment and after treatment.Results. The mean haemoglobin and haematocrit increases were 0.95 g/dL and 2.62% in the ferric group, while they were 2.25 g/dL and 5.91% in the ferrous group, respectively. A significant difference was found between the groups regarding the increase in haemoglobin and haematocrit values (P<0.05).Conclusion. Data are submitted on the good tolerability, higher efficacy, and lower cost of the ferrous preparation used in our study.

The combination of cyclophosphamide (CY) and etoposide is synergistic, spares bone marrow stem cells and can be given repeatedly in high doses without stem cell support. Thirteen patients with non-Hodgkin's lymphoma (n = 8) or Hodgkin's disease (n = 5), received high-dose chemotherapy (HDC). Median age was 32 years (24-52). Male to female ratio was 10:3. All the patients were in advanced-stage. Karnofsky score prior to HDC was 60% (range 40-90). Six patients showed primary refractoriness and 7 had resistant relapse. HDC consisted of CY 1,500 mg/m2/day and etoposide 300 mg/m2/day, both for 4 days. rhG-CSF was started 24 h after the last dose of chemotherapy as a continuous intravenous infusion at a dose of 0.01 mg/kg/day and stopped when the leukocyte count reached 1 x 10(9)/1 on 3 consecutive days. Overall, 69% (9/13) of patients responded to HDC. Four achieved CR and 5 achieved PR. Two of the patients showed disease progression. The other 2 died during the early period of HDC. Neutrophil and platelet recovery after HDC were 8 (6-16) and 10 (4-14) days, respectively. The major nonhematological toxicities were nausea-vomiting (100%) and diarrhea (61%). The median follow-up was 204 (7-600) days. Two patients relapsed 48 and 185 days after HDC. Eight patients are still alive, 7 progression free. The progression-free survival is 220 (40-285) days. In conclusion, HDC + granulocyte colony-stimulating factor (G-CSF), without stem cell support seems to be promising in refractory or resistant relapse lymphoma patients bringing the need for randomized studies to show the cost effectiveness of HDC + G-CSF compared to HDC + autologous stem cell support.