Search

Objective: The Hungarian National Registry for Philadelphia chromosome negative myeloproliferative neoplasms was used to analyse the thromboembolic events (TE) of Hungarian patients with polycythemia vera (PV)., Methods: Data from 351 JAK2 V617F-positive patients diagnosed with PV were collected online from 15 haematology centres reporting clinical characteristics, therapeutic interventions and thromboembolic events. TE events were evaluated before and after diagnosis based upon the Landolfi and Tefferi risk assessment scales., Results: TE were reported on 102 patients before diagnosis and 100 during the follow-up period. Comparing to the frequency of major arterial events before PV diagnosis, we can notice a decreasing tendency after diagnosis: from 12.3% to 2.6% (p < .00003). There was no significant change in the rate of major venous events (from 5.1% to 8.5%; p = .1134) or minor arterial events (from 11.7% to 17.4%; p = .073). Bleeding events were recorded in 5.7% of patients. Despite treatment with HU + ASA, 44 patients (43.1%) with prior TE had recurrent thromboembolic complications. The particular analysis of our data revealed a new TE scoring system based on: age, gender, previous TE and iron deficiency at the time of diagnosis., Conclusions: Our registry enables characterisation of patients with PV. The high level of recurrent TE events highlights the need for more effective and risk-adapted therapy., (© 2023 John Wiley & Sons Ltd.)

Objective: We report an extension study of patients with essential thrombocythaemia (ET) in the Hungarian Myeloproliferative Neoplasm (HUMYPRON) Registry, which demonstrated that over 6 years anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombotic events (TEs) vs hydroxyurea+aspirin., Methods: Data on patients with ET were collected through completion of a questionnaire developed according to 2008 WHO diagnostic criteria and with regard to Landolfi, Tefferi and IPSET criteria for thrombotic risk. Data were entered into the registry from 14 haematological centres. TEs, secondary malignancies, disease progression and survival were compared between patients with ET treated with anagrelide (n = 116) and with hydroxyurea+aspirin (n = 121)., Results: Patients were followed for (median) 10 years. A between-group difference in the number of patients with TEs was observed (25.9% anagrelide vs 38.0% hydroxyurea+aspirin; P = .052). Minor arterial events were more frequently reported in the hydroxyurea+aspirin group (P < .001); there were marginally more reports of major arterial events in the anagrelide group (P = .049). TE prior to diagnosis was found to significantly influence TE incidence (P > .001). Progression-free survival (P = .004) and survival (P = .001) were significantly increased for the anagrelide group vs hydroxyurea+aspirin., Conclusions: Anagrelide reduced TEs, and increased progression-free and overall survival vs hydroxyurea+aspirin over (median) 10 years., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Daratumumab is a human anti-CD38 monoclonal antibody used in the treatment of refractory and relapsed multiple myeloma. We investigated the efficacy and safety of daratumumab therapy in a real-world setting. Ninety-nine Hungarian patients were included; 48 received monotherapy, while lenalidomide and bortezomib combinations were administered in 29 and 19 cases, respectively. Overall response rate was assessable in 88 patients, with 12 complete, 10 very good partial, 34 partial, and seven minor responses. At a median duration of follow-up of 18.6 months, median progression-free survival (PFS) among all patients was 17.0 months. These values were inferior in the bortezomib combination and monotherapy groups. Patients with early-stage disease (ISS1) had better survival results than those with stage 2 or 3 myeloma (p = 0.009). Heavily pretreated patients had inferior PFS compared to those with 1-3 therapies (p = 0.035). Patients with impaired renal function had PFS results comparable with those having no kidney involvement. There were 10 fatal infections, and the most frequent adverse events were mild infusion-associated reactions and hematologic toxicities. Our results confirm that daratumumab is an effective treatment option for relapsed/refractory MM with an acceptable safety profile in patients with normal and impaired renal function.

Introduction: The establishment and operation of disease registry can be used to collect data on epidemiology cases. In addition, the registry can help to work out medical and health economical and political decisions for longer term., Aim: The aim of the authors was to collect and analyse data of patients with Philadelphia negative neoplasia in Hungary and draw conclusions about the basic types and features of the relevant disease., Method: An online electronic data collection system has been established, based on the permission of the Regional and Institutional Committee of Science and Research Ethics obtained in April 8, 2013. Data collection has been initiated by hematology centres in Hungary. In addition to collection of the epidemiologic data, blood and bone marrow analysis data have been collected, as well. Also, based on cardiovascular factors, risk stratification has been established. Finally, the authors have investigated the method and practice of patient treatment in Hungary., Results: Data of 901 patients from 15 Hungarian haematology centres have been recorded up to the date of June 30, 2015. After clarification of the data, 426 polycythaemia vera, 350 essential thrombocythaemia and 82 myelofibrosis cases were used for analysis., Conclusions: An online registry has been established which helps to clarify and analyse the basic features of certain medical cases and their treatment in Hungary. Including additional medical centres could help to improve the accuracy of medical analysis.

Introduction: Screening for iron deficiency, which affects a significant proportion of the population, is a burning issue in the health care system., Aim: The aim of the authors was to examine whether low mean cell hemoglobin concentration measured by automated hematology analyzers is a suitable screening parameter for iron deficiency., Method: The data for this study included a total of 247,705 complete blood counts and 10,840 tests with different parameters of iron metabolism. Patients were evaluated at Somogy County Kaposi Mór Teaching Hospital during a period of 30 months between January 1, 2013 and June 30, 2015. Low cell hemoglobin values were analyzed with iron metabolism parameters measured simultaneously., Results: A total of 830 patients whose iron metabolism parameters were measured simultaneously had low mean cell hemoglobin (<28pg). Of the 830 patients, 679 (82%) had both low mean cell hemoglobin and iron deficiency, while in 126 hemodialysed patients (15%), 8 patients with myelofibrosis, and 5 patients with rheumatic arthritis had low mean cell hemoglobin without iron deficiency. In the remaining 6 patients the cause of low mean cell hemoglobin or iron deficiency was not identified., Conclusions: Based on these findings the authors conclude that mean cell hemoglobin may be a reliable screening marker for iron deficiency.

Unlabelled: Over the past few decades, the occurrence of adult onset non-Hodgkin's lymphoma has significantly increased. The patient population involved is very heterogeneous, with different clinical and morphological manifestations. In addition to the most typical nodal involvement, extra-nodal manifestations are also frequent, affecting, most often, the gastrointestinal tract, the central nervous system and the skin. The treatment strategy for non-Hodgkin's lymphoma has changed over the past decade: chemo-immunotherapy has largely taken over surgical intervention, the dominant treatment option of the past., Methods: The authors present their experience with 48 patients with non-Hodgkin's lymphoma, affecting the gastrointestinal tract, treated in Kaposvár, in the Kaposi Mór Teaching Hospital and in Gyula, in the Pándy Kálmán County Hospital. Demography: 27 female, 21 male; mean age: 67.8 years. Localization, pathological classification and the international prognostic index (IPI) have been analysed and correlated with the clinical response to different therapeutic strategies., Results: The most frequently involved GI organ was the stomach ( n = 26), with the dominant histological type of diffuse large B-cell lymphoma. Fourty-six patients received chemo-immunotherapy, 6 received radiotherapy, 3 patients were primarily treated with Helicobacter pylori eradication therapy, and 4 patients were referred for stomach resection. A complete remission was achieved in 68% of the patients, a partial remission in 13%, while 19% did not show clinical response. Based on the international prognostic index, the majority of the patients fulfilled criteria of low or high intermediate risk categories, with an IPI average of 2.68. Patients with upper gastrointestinal tract involvement carried the best prognosis (IPI: 2.0); at the same time, patients with stomach lymphoma achieved the highest rate of remission (73%)., Conclusions: With chemo-immunotherapy the chances of a complete remission have significantly improved over the past decade, thus a significant portion of non-Hodgkin's lymphomas involving the gastrointestinal tract can be cured. IPI index represents the most recognised indicator for assessing the prognosis of non-Hodgkin's lymphoma. Patients who achieved complete remission had the lowest prognostic index in this cohort; nevertheless, numerous data indicate that factors other than the IPI can also have an impact on patients' response to treatment.

Introduction: The neutropenic patient's infections are challenging problems for modern medicine. The increasing number of iatrogenic neutropenia, the invasive procedures and the growing resistance to antibiotics and antimycotics make the treatment of sepsis difficult. The aim of this study was to analyse the frequency and mortality of neutropenic infections in hematologic patients., Methods and Results: In the department of the authors 146 patients with sepsis were diagnosed out of 2173 treated patients in 24 months (67/1000 patients). 78 of them were neutropenic (absolute neutrophil count below 0,5 G/I) and 63 were severe neutropenic (absolute neutrophil count below 0,1 G/I). Sepsis occurred on the 10-11th day of neutropenia. 45% of positive hemoculture were caused by Gram positive bacteria, 30% by Gram negative bacteria, 10% by fungi and 15% were polymicrobic infections. The overall mortality was 36%, but in the severe neutropenic group it was about 60%., Conclusions: These results show that despite of the use of new, broad spectrum of antibiotics and antimycotics in neutropenic patients with sepsis, it is difficult to treat these patients.