Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience.

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.

The Turkish experience with therapeutic plasma exchange: A national survey.

Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.

Can Rotational Thromboelastometry be a New Predictive Tool for Retinal Vein Occlusion Development?

PURPOSE: To evaluate clotting dynamics by a new tool called rotational tromboelastometry (ROTEM) in retinal vein occlusion. MATERIALS AND METHODS: Thirty-six patients who were diagnosed with retinal vein occlusion and 43 age and sex matched healthy controls were included in this study. Diabetes and use of anticoagulant therapy were exclusion criteria. All study participants underwent detailed ophthalmologic and systemic medical examination, including blood pressure measurement, hemoglobin-hematocrit levels, platelet count, coagulation parameters including prothrombin time, activated partial thromboplastin time, fibrinogen levels, and D-dimer levels. Peripheral blood samples were collected and analyzed with ROTEM Coagulation Analyzer (Tem International, Munich, Germany). RESULTS: The RVO patients and controls did not differ with respect to age, sex, hemoglobin, hematocrit, platelet numbers, prothrombin time, activated partial thromboplastin time, fibrinogen levels, D-dimer levels, and glucose levels. When extrinsic thromboelastometry results were analyzed, RVO patients showed a significantly decreased clotting time (76.5 ± 15.0 vs. 95.0 ± 21 s, respectively; p = 0.01) and clot formation time (83.3 ± 22 vs. 99.7 ± 24s; p = 0.01) as compared with healthy controls. Other ROTEM parameters did now show any difference between two groups. CONCLUSION: Patients with retinal vein occlusion showed faster clotting time and shorter clotting formation time as compared with healthy controls. ROTEM detects the altered clotting dynamics and may be a useful tool to elucidate the disease pathophysiology. Further studies are needed to investigate if it can be used as a screening test for individuals who are under risk to develop RVO or as a first step test to evaluate hypercoagulable state in RVO.

Peripheral blood stem cell collection for allogeneic hematopoietic stem cell transplantation: Practical implications after 200 consequent transplants.

BACKGROUND: Proper stem cell mobilization is one of the most important steps in hematopoietic stem cell transplantation (HSCT). The aim of this paper is to share our 6 years' experience and provide practical clinical approaches particularly for stem cell mobilization and collection within the series of more than 200 successive allogeneic HSCT at our transplant center. SUBJECTS & METHODS: Two hundred and seven consecutive patients who underwent allogeneic peripheral blood stem cell transplantation were included in this study. Age, sex, weight, complete blood counts, CD34+ cell counts, total collected amount of CD34+ cells, CD34+ cells per 10l processed, mobilization failure and adverse events were reviewed. RESULTS: Median age was 40.2±12.9 (21-68) years and 46.4±13.4 (17-67) years for donors and patients, respectively. The number of donors who had undergone adequate CD34+ cell harvesting and completed the procedure on the fourth day was 67 (32.8% of all patients). Only 12 patients required cell apheresis both on day 5 and 6. Apheresis was completed on day 4 and/or day 5 in 94.2% of all our donors. There was no significant association between CD34+ stem cell volume and age, gender and weight values of donors. Mobilization failure was not seen in our series. CONCLUSIONS: G-CSF is highly effective in 1/3 of the donors on the 4th day in order to collect enough number of stem cells. We propose that peripheral stem cell collection might start on day 4th of G-CSF treatment for avoiding G-CSF related side effects and complications.

Comparison of Rotational Thromboelastography Findings in Pseudoexfoliation Syndrome Patients and Healthy Controls.

PURPOSE: Rotational thromboelastography (ROTEM) is a useful test for studying the characteristics of fibrin clot formation. As patients with pseudoexfoliation (PEX) have an increased risk for thrombotic events, clot-forming dysfunction may play a role. The aim of this study is to compare ROTEM findings in PEX syndrome patients with age-matched and sex-matched healthy controls. PATIENTS AND METHODS: A total of 21 patients with PEX syndrome and 22 age-matched and sex-matched healthy controls were included. All study participants underwent detailed ophthalmologic and systemic medical examination, including blood pressure measurement, hemoglobin-hematocrit levels, platelet count, coagulation parameters including prothrombin time, activated partial thromboplastin time, fibrinogen levels, and D-dimer levels. Peripheral blood samples were collected and analyzed with ROTEM Coagulation Analyzer. RESULTS: The mean age of patients with PEX and controls was 66.5±8.3 and 65.9±9.5 years, respectively (P=0.7). The 2 groups did not differ with respect to age, sex, hemoglobin, hematocrit, platelet numbers, prothrombin time, activated partial thromboplastin time, fibrinogen levels, D-dimer levels, and glucose levels. When extrinsic thromboelastometry results were analyzed, PEX patients showed a significantly decreased clotting time when compared with healthy controls (79.8 vs. 98.0 s; P=0.01), indicating faster clot formation. Other ROTEM parameters did now show any difference between the 2 groups. CONCLUSIONS: PEX patients showed faster clotting time when compared with healthy controls. This fibrin clot formation dysfunction may lead to vascular thrombotic events in these patients. Further studies are needed to elucidate the exact underlying mechanism of thrombosis seen in PEX patients.

The Role of Azacitidine in the Treatment of Elderly Patients with Acute Myeloid Leukemia: Results of a Retrospective Multicenter Study.

OBJECTIVE: In this study, we aimed to investigate the efficacy and safety of azacitidine (AZA) in elderly patients with acute myeloid leukemia (AML), including patients with >30% bone marrow (BM) blasts. MATERIALS AND METHODS: In this retrospective multicenter study, 130 patients of ≥60 years old who were ineligible for intensive chemotherapy or had progressed despite conventional treatment were included. RESULTS: The median age was 73 years and 61.5% of patients had >30% BM blasts. Patients received AZA for a median of four cycles (range: 1-21). Initial overall response [including complete remission (CR)/CR with incomplete recovery/partial remission] was 36.2%. Hematologic improvement (HI) of any kind was documented in 37.7% of all patients. HI was also documented in 27.1% of patients who were unresponsive to treatment. Median overall survival (OS) was 18 months for responders and 12 months for nonresponders (p=0.005). In the unresponsive patient group, any HI improved OS compared to patients without any HI (median OS was 14 months versus 10 months, p=0.068). Eastern Cooperative Oncology Group performance status of <2, increasing number of AZA cycles (≥5 courses), and any HI predicted better OS. Age, AML type, and BM blast percentage had no impact. CONCLUSION: We conclude that AZA is effective and well tolerated in elderly comorbid AML patients, irrespective of BM blast count, and HI should be considered a sufficient response to continue treatment with AZA.

Utility of flow cytometric κ and λ light chain analysis of peripheral blood.

PURPOSE: Monoclonality in the peripheral blood can be shown by flow cytometric analysis of kappa (κ) and lambda (λ) light chain ratio of B lymphocytes. We aimed to show the utility of this method in patients with unknown causes of lymphadenopathy and/or splenomegaly. METHODS: This method was performed in 81 adult patients with undefined causes of lymphadenopathy and splenomegaly. RESULTS: 18 (22%) of these patients had clonality and all of them were diagnosed as B cell lymphoma later. None of the patients with benign causes had clonality in the peripheral blood. We could not find any relationship between presence of clonality and type and stage of lymphoma and bone marrow involvement. CONCLUSION: This method is easy to perform, cheap and non-invasive and yet it can give valuable information about the malignant nature of a suspected disease. If there is a sign of clonality in the peripheral blood, more invasive diagnostic procedures should be performed rather than watch and wait.

The utility of thromboelastometry in prophylactic platelet transfusion for hematological malignancies.

There is still debate on how platelet transfusions should be used to prevent severe bleeding. The aim of our study is to assess the clinical efficacy of thromboelastometry in reducing number of prophylactic platelet transfusions in patients with hematological malignancies. One hundred hematological malignancy patients were included in the study. Six units random donor platelets (RDPs) was given to the first group, three units RDPs was given to the second group, one unit single donor platelets (SDPs) was given to the third group, and 1/2 unit SDPs was given to the fourth group. Before and 15 minutes after transfusion, rotation thromboelastometry (ROTEM) was performed (Pentapharm GmbH, Munich, Germany). ROTEM(®) parameters did not show any statistical difference between 'low dose' and 'high dose' random or single donor platelet transfusions. Therefore, low dose platelet transfusion can be considered because of its reduced adverse transfusion reactions and economic burden.

Pleural effusion: a rare side effect of nilotinib-a case report.

Pleural effusion, as a side effect of tyrosine kinases, may be seen as most commonly associated with dasatinib and very rarely seen with nilotinib. In this report we present a chronic phase of CML case that was treated with nilotinib due to imatinib (Gleevec) allergy and had pleural effusion with nilotinib at 5th year of treatment. If pleural effusion develops in patients taking nilotinib and if this effusion is exudative and lymphocyte predominant, after ruling out pulmonary and cardiac etiologies, it must be associated with nilotinib; according to stage of effusion drug should be discontinued and/or steroid should be started and/or surgery should be performed.

Blastic plasmacytoid dendritic cell leukemia successfully treated by autologous hematopoietic stem cell transplantation to a remission of 48-month duration.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare subtype of AML characterized by the clonal proliferation of precursors of plasmacytoid dendritic cells. It presents with an aggressive behavior. The clinical findings include cytopenia, particularly thrombocytopenia. Although it responds well to chemotherapy initially, the relapse is a rule and prognosis is very poor. There is limited data published in the literature, making it very problematic to define the biological and clinical features, hence, the appropriate therapeutic approach. There are various treatment methods such as multiagent chemotherapy based on ALL or AML and/or hematopoietic stem cell transplantation. However, none of them is approved as a standard therapy. From this point of view, we herein report a 20-year-old case at onset of a leukemic form of BPDCN who survived 48 months after autologous hematopoietic stem cell transplantation.