A potentially new thromboembolic event scoring system in polycythaemia vera patients: An audit of the Hungarian Philadelphia negative chronic myeloproliferative neoplasia register.

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.

Two-Year Event-Free Survival Prediction in DLBCL Patients Based on In Vivo Radiomics and Clinical Parameters.

Purpose: For the identification of high-risk patients in diffuse large B-cell lymphoma (DLBCL), we investigated the prognostic significance of in vivo radiomics derived from baseline [18F]FDG PET/CT and clinical parameters. Methods: Pre-treatment [18F]FDG PET/CT scans of 85 patients diagnosed with DLBCL were assessed. The scans were carried out in two clinical centers. Two-year event-free survival (EFS) was defined. After delineation of lymphoma lesions, conventional PET parameters and in vivo radiomics were extracted. For 2-year EFS prognosis assessment, the Center 1 dataset was utilized as the training set and underwent automated machine learning analysis. The dataset of Center 2 was utilized as an independent test set to validate the established predictive model built by the dataset of Center 1. Results: The automated machine learning analysis of the Center 1 dataset revealed that the most important features for building 2-year EFS are as follows: max diameter, neighbor gray tone difference matrix (NGTDM) busyness, total lesion glycolysis, total metabolic tumor volume, and NGTDM coarseness. The predictive model built on the Center 1 dataset yielded 79% sensitivity, 83% specificity, 69% positive predictive value, 89% negative predictive value, and 0.85 AUC by evaluating the Center 2 dataset. Conclusion: Based on our dual-center retrospective analysis, predicting 2-year EFS built on imaging features is feasible by utilizing high-performance automated machine learning.

Anagrelide influences thrombotic risk, and prolongs progression-free and overall survival in essential thrombocythaemia vs hydroxyurea plus aspirin.

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.

[Hungarian Philadelphia negative chronic myeloproliferative neoplasia registry. Evaluation of the Polycythemia vera patients]. / Philadelphia-negatív krónikus Myeloproliferativ Neoplasia Magyarországi Regiszter. Polycythaemia verás betegeink adatainak elemzése.

Intruduction and aim: The Hungarian National Registry for Philadelphia chromosome negative myeloproliferative neoplasms has been developed. The aim of the recent study is to assess the clinical characteristics of Hungarian patients with polycythemia vera. METHOD: Data of 351 JAK2V617F and exon 12 mutation positive polycythemia vera patients were collected online from 15 haematology centres reporting epidemiologic, clinical characteristics, diagnostic tools, therapeutic interventions, thromboembolic complications, disease transformations. Vascular events prior to and after diagnosis were evaluated upon the Landolfi risk assessment scale. RESULTS: 116 thromboembolic events were reported in 106 PV patients prior to diagnosis and 152 occasions in 102 patients during follow-up. The frequency of major arterial events were significantly reduced (p<0.0001) and the minor venous events were significantly elevated (p<0.0001) after the diagnosis. Major hemorrhagic complications were found in 25 and transformation in 26 cases. CONCLUSIONS: Our registry allows to collect and evaluate the features of patients with polycythemia vera. The Landolfi risk stratification was proven to be useful. Based on evaluated data, accuracy of diagnostic criteria and compliance to risk-adapted therapeutic guidelines are needed. Orv Hetil. 2017; 158(23): 901-909.

[An audit of the Hungarian Philadelphia negative chronic myeloproliferative neoplasia registry. Evaluation of the essential thrombocythaemic patients]. / Philadelphia-negatív krónikus Myeloproliferativ Neoplasia Magyarországi Regiszter. Essentialis thrombocythaemiás betegeink adatainak elemzése.

INTRODUCTION: In order to establish and use a national registry, several Hungarian hematology centers collected data of myeloproliferative neoplasia patients. AIM: The recent publication is an analysis of the data of registered essential thrombocythaemic patients. METHOD: an online electronic registry has been established, using 2008 World Health Organization's diagnostic criteria and thrombotic risk was evaluated according to Landolfi stratification. RESULTS: Data of 350 essential thrombocythaemic patients from 15 Hungarian hematology centers entered up to the date of June 30, 2015 were used for analysis. Patients were followed up to (median) 6 years. The epidemiologic data (age, gender) and thrombotic events prior and after the diagnosis, were similar to the literature. The thrombotic events of anagrelide treated patient (n = 139) and the hydroxyurea + aspirin treated patients (n = 141) have been compared. The major arterial and venous events were similar between the groups, but there were fivefold less minor arterial and venous events in the anagrelide group (p<0.001). Thrombotic incidence after diagnosis were influenced only by medication and thrombotic events before the diagnosis. CONCLUSIONS: Anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombosis, vs hydroxyurea + aspirin. Despite of the treatment the risk of thrombotic events after diagnosis remained high, and was significantly increased in patients with thrombosis before diagnosis. Orv. Hetil., 2017, 158(3), 111-116.

Anagrelide reduces thrombotic risk in essential thrombocythaemia vs. hydroxyurea plus aspirin.

OBJECTIVE: To evaluate the reduction in thrombotic events (TE) in patients with essential thrombocythaemia (ET) treated with anagrelide versus hydroxyurea + aspirin (HU + ASA). METHODS: A questionnaire was developed using 2008 WHO diagnostic criteria, and thrombotic risk factors were stratified according to Landolfi criteria. Through questionnaire completion, clinicians at Hungarian haematological centres entered data into the Hungarian MPN Registry on patients with myeloproliferative neoplasms. Based on ET registry data, TEs in anagrelide-treated patients (n = 139) were compared with HU + ASA-treated patients (n = 141). RESULTS: Patients were followed up for (median) 6 yr. TEs were reported in significantly fewer anagrelide-treated patients versus HU + ASA (15.1% versus 49.6%; P < 0.001). Numbers of major arterial and major venous events were similar between the groups, although there were over fivefold more minor arterial and minor venous events in the HU + ASA group (P < 0.001). While median age at diagnosis was older and length of follow-up shorter in the HU + ASA group (P < 0.05), this did not influence TE incidence; medication and TE before diagnosis only influenced TE incidence. CONCLUSIONS: Anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous TEs versus HU + ASA over 6 yr. Risk of TE after diagnosis was significantly increased if the patient had TE before diagnosis.

[Low mean cell hemoglobin is a reliable marker for iron deficiency screening]. / Az alacsony átlagos vörösvértest-hemoglobin alkalmas a vashiány szurésére.

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.

[Imatinib therapy in chronic myeloid leukemia]. / Krónikus myeloid leukaemiás betegeink imatinibkezelése.

UNLABELLED: Chronic myeloid leukemia is a malignant clonal alteration of the pluripotent hemopoietic stem cell. The genetic hallmarks of the disease are the t(9,22) (Philadelphia chromosome), registered by conventional cytogenetics in more than 90% of all chronic myeloid leukemia cases and the active tyrosine kinase protein encoded by bcr-abl fusion gene. The constitutively active tyrosine kinase is currently accepted to be the cause of chronic myeloid leukemia. The introduction of imatinib has considerably changed the treatment of chronic myeloid leukemia. Prior studies demonstrated high rates of cytogenetic responses in all phases of the disease. METHODS: The authors evaluated the cytogenetic and molecular responses of 21 chronic phase chronic myeloid leukemia patients who were consecutively admitted to their center. 13 of them were primarily treated with imatinib, and the other 7 were heavily pretreated with interferon alpha, cytarabine, all-trans-retinoic acid. Hydroxyurea pretreatment was routinely introduced in all patients until complete hematologic remission. Peripheral blood sample in every 3 months were collected for quantitative real-time polymerase chain reaction, and bone marrow aspirate in every 6 months for conventional cytogenetics. RESULTS: Hematologic remission could have been achieved with hydroxyurea pretreatment in each patient. Complete cytogenetic remission at the 6th month and major molecular response at the 12th month were observed in each patient. CONCLUSIONS: Imatinib treatment caused complete cytogenetic response and major molecular response in each chronic phase chronic myeloid leukaemia patient in our group. Hydroxyurea might have some effect on the rapid and deep cytogenetic and molecular responses, observed in the primary imatinib-treated group.

[Neutropenia and sepsis in hematologic patients]. / Neutropenia és szepszis a hematológiai osztályon: a Kaposi Mór Oktató Kórház tapasztalatai.

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.