The old and new thrombotic risk factors in essential thrombocythemia patients

Essential Thrombocythemia (ET) is one of the classical Philadelphia negative myeloproliferative neoplasms. Despite the chronic course of the disease, the ET patients had an inferior survival compared to the general population, because of higher mortality, mainly due to thrombotic complications. Besides well known old risk factors, such as old age (>60 year) and previous history of thrombosis, still some new ones appeared. In 2005 the JAK2V617F point mutation was found in 50-60% of ET patients. An increased risk of thrombosis in patients with this mutation has been reported. In this study there is a summary of old and new risk factors which may contribute to thrombotic complications in ET patients

Dried Blood Spot (DBS) test for diagnosis of Gaucher and Niemann-Pick diseases

In this paper there is a presentation of early results of the DBS test as a diagnostic for Gaucher and Niemann-Pick diseases

Primary central nervous system lymphoma as a neurosurgical problem

Primary central nervous system lymphoma (PCNSL) comprises around 3–5% of primary central nervous system (CNS) tumours and around 1% of all non-Hodgkin lymphoma (NHL). Diffuse large B-cell lymphoma (DLBCL) is the most common histological type. High effectiveness of chemo- and radiotherapy for PCNSL regrettably does not eliminate significant risks of recurrence for CNS tumours. That results in higher interest in other treatment options, including surgical procedures. PCNSL remains in the scope of interest for many specialists and neurosurgeons seem to play a more important role

Rola PF4 (chemokiny CXCL4) w powstawaniu skrzepu

Platelet factor 4 (PF4) is released from platelet α granules during activation process. It takes part in clot formation. Physiological concentration of PF4 is essential not only for thrombus formation but also for the anticoagulant potency of heparin. This review is based on experiments performed by one of coauthors.Czynnik płytkowy 4 (PF4) uwalniany jest z ziarnistości α krwinek plytkowych podczas ich aktywacji. Uczestniczy w powstawaniu skrzepu. Prawidłowe stężenie PF4 warunkuje nie tylko efektywność procesów krzepnięcia krwi, ale również skuteczność terapeutycznych dawek heparyny. Praca niniejsza opiera się w dużej mierze na pracach doświadczalnych jednej z współautorek

Skuteczne leczenie ibrutynibem chorego na przewlekłą białaczką limfocytową z obecnością delecji 17p – opis przypadku

W pracy przedstawiono przypadek 62-letniego chorego na PBL z obecnością delecji 17p, u którego zastosowanie immunochemioterapii nie przyniosło oczekiwanego efektu. Dopiero leczenie ibrutynibem doprowadziło do remisji częściowej choroby (zmniejszenia limfadenopatii, normalizacji obrazu morfologii krwi)

Autoewaluacja w szkole promującej zdrowie. Metody i narzędzia.

Publikacja omawia definicję, model i standardy szkoły promującej zdrowie oraz metody autoewaluacj

Haemophagocytic lymphohistiocytosis: case series. Serum ferritin level as an indicator of treatment effectiveness

Introduction: Haemophagocytic lymphohistiocytosis (HLH) is a medical condition associated with over-activation of the immune system. HLH results from the inactivity of NK cells and uncontrolled activity of cytotoxic T lymphocytes and macrophages with the massive cytokine response.[1] Cytohistological examinations might show hemophagocytes in different tissues (e.g., bone marrow). Exceptionally high level of ferritin is often observed in the laboratory findings. Due to aggressive course of the disease mortality rate is extremely high. Material and methods: We presented three patients with acquired form of HLH treated successfully in Heaematooncology Department of Medical University of Lublin from September 2018 to April 2021. Results: In the first patient, HLH developed during pregnancy. Patient 2 was first hospitalized in the Intensive Care Unit (ICU) and 10 therapeutic plasma exchanges (TPE) were carried out. In both patients, stabilization -of ferritin levels and remission of the disease were achieved soon after the application of the treatment according to the HLH-2004 protocol. Case 3 presents a patient in whom HLH was induced by EBV and SARS CoV-2 infections. In the first stage of the treatment, the patient received drugs according to the HLH-2004 protocol, but the serum ferritin did not normalize. The reinfection with SARS CoV-2 virus occurred again in the course of the treatment. Due to the disease resistance and inability to continue the HLH-2004 protocol, it was decided to start ruxolitinib therapy, which resulted in stabilization of the serum ferritin and improvement of the general condition. The patient was qualified for allogeneic bone marrow transplantation. Conclusions: HLH is a difficult and interdisciplinary diagnostic and therapeutic problem. It is essential to popularize knowledge about fast and targeted diagnostics. Among laboratory finding, the ferritin concentration seemed to be particularly helpful as a predictor of treatment effectiveness. Proper diagnosis and treatment implemented as early as possible could save patients’ lives.Introduction: Haemophagocytic lymphohistiocytosis (HLH) is a medical condition associated with the over-activation of the immune system. HLH results from the inactivity of natural killer cells and uncontrolled activity of cytotoxic T lymphocytes and macrophages, with a massive cytokine response. Cytohistological examinations might show haemophagocytes in different tissues (e.g., bone marrow). Among laboratory findings especially(particularly) high ferritin level is often seen. Due to the aggressive course of the disease mortality rate is extremely high. Case reports: The study presented three patients with an acquired form of HLH treated successfully in the Haemato-Oncology Department Medical University of Lublin from September 2018 to April 2021. In case 1 HLH developed during pregnancy. Patient 2 was first hospitalized in the Intensive Care Unit and 10 therapeutic plasma exchanges were carried out. In both patients, stabilization of ferritin levels and remission of the disease were achieved soon after the application of treatment according to the HLH-2004 protocol. Case 3 presents a patient in whom HLH was induced by Epstein-Bárr virus and severe acute respiratory syndrome coronavirus type SARS-CoV-2 infections. In the first stage of treatment, the patient received drugs according to the HLH-2004 protocol, but the serum ferritin did not normalize. The reinfection with (SARS-CoV-2) virus occurred again during the treatment. Due to the disease resistance and the inability to continue the HLH-2004 protocol, it was decided to start ruxolitinib therapy, which resulted in the stabilization of the serum ferritin and improvement of the general condition. The patient was qualified for allogeneic bone marrow transplantation. Conclusions: HLH is a difficult and interdisciplinary diagnostic and treatment problem. It is necessary to popularize knowledge about fast and targeted diagnostics. Among laboratory finding the ferritin concentration seemed to be especially helpful as a predictor of treatment effectiveness. Proper diagnosis and treatment introduced as early as possible could save patients’ life

Expression of immune checkpoints on peripheral blood cells in patients with prostate cancer

Introduction. The first clinical trials of the treatment of patients with prostate cancer with checkpoint inhibitors show that only a few patients benefit from this type of treatment. This implies the need to find predictive biomarkers that would help identify patients for whom treatment with checkpoint inhibitors could be effective. Our study aimed to assess the level of PD-1, PD-L, and CTLA-4 expression on peripheral blood mononuclear cells of patients with primary prostate cancer and to demonstrate their applicability in clinical practice.  Material and methods. Fifty men with primary prostate cancer were enrolled in the study. The control group consisted of 20 healthy men. The material for the study was peripheral blood from which mononuclear cells were isolated by flow cytometry, and the expression of PD-1, CTLA-4, and PD-L1 on them was assessed.  Results. High PD-L1 expression on lymphoid dendritic cells has been demonstrated in patients with prostate cancer in comparison to the control group (p = 0.015) and high PD-L1 expression has been demonstrated in the following groups: high risk (p = 0.026), T2/T3 (p = 0.011), and Gleason 7 (p = 0.027). There was no high PD-1 expression on T lymphocytes among patients with prostate cancer in comparison to the control group, but positive correlations were found between PD-1 expression on CD3+ T cells and PSA (p = 0.049), risk group (p = 0.002), and TNM (p = 0.050). Low CTLA-4 expression was found on CD3+ lymphocytes among patients with prostate cancer in comparison to the control group (p = 0.006).  Conclusions. Several groups of patients with prostate cancer have been identified, showing high PD-L1 and PD-1 expression on peripheral blood mononuclear cells, and a relationship between PD-1 and PD-L1 expression and the tumor aggressiveness potential has been demonstrated. This means that the assessment of PD-1 and PD-L1 expression can be used as a prognostic and predictive biomarker in prostate cancer

JAK2 mutation status, hemostatic risk factors and thrombophilic factors in essential thrombocythemia (ET) patients

The recently discovered JAK2 V617F point mutation, found in 50–60% of ET patients, has been reported to be associated with a higher risk of thrombotic events. In this study, we explored if JAK2 V617F mutation, or coexisting thrombophilic and hemostatic risk factors, contributed to these complications. We examined 32 patients with ET, and looked for pathogenetic JAK2 V617F mutation and prothrombotic genes mutations: factor V Leiden, prothrombin and MTHFR. We also evaluated plasma levels of fibrinogen, factors VIII and XII, AT, protein C, protein S and serum level of homocysteine. Urokinase concentration was assessed in patients’ plasma as well as platelet lysates. There was no difference in the number of thrombotic complications between ET patients with and without JAK2 mutation. However, we found a number of thrombophilic and hemostatic risk factors that could contribute to thrombotic complications in ET patients. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 2, pp. 267–271