What factors determine the pregnancy outcome in patients with essential thrombocythemia?

Treatment of essential thrombocythemia (ET) is particularly challenging in pregnancy due to the increased risk of thromboembolic complications. Therefore, the use of antithrombotic regimens are recommended in pregnant women with ET. The study included 52 pregnancies in 27 patients diagnosed with ET, who were treated in Department of Haematology.

Treatment of Essential Thrombocythemia with Anagrelide Is Associated with an Increased Risk of Worsened Kidney Function.

Background And Purpose: When choosing a cytoreduction method for patients suffering from essential thrombocythemia (ET), it is important to know the safety profile of the medicine used. Few articles have been published about the effects of hydroxycarbamide (hydroxyurea, HU) and anagrelide (ANA) on renal function in ET patients. This study is the largest analysis of nephrotoxicity of cytoreductive drugs used in ET therapy so far, which additionally includes risk factors for the progression of kidney disease and coexisting genetic mutation.

The pathogenesis and available prevention options in patients with diabetic thrombophilia.

Diabetes mellitus (DM), a growing health problem itself, is accompanied by an increased risk of cardiovascular and thrombotic complications. The imbalance between coagulation and fibrinolysis processes observed in patients with diabetes may be defined as diabetic thrombophilia. Several mechanisms are involved in the hypercoagulability state in diabetics, including endothelial cell damage, altered platelet structure and function, increased microparticle formation, different structure of fibrin clots, disturbances in the activity of coagulation factors, fluctuations in the concentrations of fibrinolysis activators and inhibitors, and qualitative changes of proteins due to glycation and oxidation processes.

Constitutional mutations of the CHEK2 gene are a risk factor for MDS, but not for de novo AML.

CHEK2 plays a key role in cellular response to DNA damage, and also in regulation of mitosis and maintenance of chromosomal stability. In patients newly diagnosed with myelodysplastic syndrome (MDS, n = 107) or acute myeloid leukemia (AML, n = 117) congenital CHEK2 mutations (c.444 + 1G > A, c.

Stage of liver fibrosis in patients with congenital bleeding disorders and infected with hepatitis C virus.

Introduction: Hepatitis C virus (HCV) is the major cause of chronic liver disease in patients with hemophilia. However, since liver biopsy should not be routinely used in these patients, the accurate assessment of the stage of fibrosis has been limited so far.

Objectives: The aim of this study was to determine the stage of liver fibrosis in HCV‑infected patients with hemophilia by using noninvasive methods of fibrosis assessment, and to analyze the influence of risk factors on liver fibrosis.

Peripheral circulatory disorders in essential thrombocythemia.

A significant number of patients with essential thrombocythemia (ET) complain of symptoms including distal parts of the extremities (e.g., paresthesias or Raynaud's phenomenon).

Co-Infection of the Hepatitis C Virus With Other Blood-Borne and Hepatotropic Viruses Among Hemophilia Patients in Poland.

Background: The prevalence of HCV infection in people with hemophilia is substantially higher than that in the general population (63% - 98%). Multiple transfusions and substitutive therapy have also been linked to a high risk of HBV and HIV transmission. However, the prevalence of other blood-borne viral infections in this population is less well known.

Current Possibilities to Assess the Degree of Liver Fibrosis in Patients with Haemophilia Infected with HCV--Review.

Haemophilia is an entity, wherein the HCV infection rate is greater than in the general population and ranges between 70-90%. The majority of HCV infections were acquired by hemophiliacs in the 1980s, by the use of infected cryoprecipitate or fresh frozen plasma preparations. It is therefore highly likely that many of them, more than twenty years after the infection, have developed advanced liver disease.

Altered plasma fibrin clot properties in essential thrombocythemia.

Patients with increased thromboembolic risk tend to form denser fibrin clots which are relatively resistant to lysis. We sought to investigate whether essential thrombocythemia (ET) is associated with altered fibrin clot properties in plasma. Ex vivo plasma fibrin clot permeability coefficient (Ks), turbidimetry and clot lysis time (CLT) were measured in 43 consecutive patients with ET (platelet count from 245 to 991 × 10(3)/µL) and 50 control subjects matched for age, sex and comorbidities.

A risk of essential thrombocythemia in carriers of constitutional CHEK2 gene mutations.

Germline mutations of the CHEK2 gene have been reported in some myeloid and lymphoid malignancies, but their impact on development of essential thrombocythemia has not been studied. In 16 out of 106 (15.1%) consecutive patients, newly diagnosed with essential thrombocythemia, we found one of four analyzed CHEK2 mutations: I157T, 1100delC, IVS2+1G>A or del5395.

[Mastocytosis--diagnostic criteria and treatment].

Mastocytosis is a heterogeneous group of rare diseases characterized by the proliferation and accumulation of mast cells in one or more organs such as the skin, bone marrow, liver, spleen, and lymph nodes. According to the WHO classification, mastocytosis is divided into seven subvariants. The symptoms are associated with mediator release and impaired organ function due to infiltration by neoplastic mast cells.

31P MRS analysis of the phospholipid composition of the peripheral blood mononuclear cells (PBMC) and bone marrow mononuclear cells (BMMC) of patients with acute leukemia (AL).

The aim of this study was to evaluate the phospholipid concentration in acute leukemia (AL) blast cells from peripheral blood (PBMC) and bone marrow (BMMC). In vitro 31P Nuclear Magnetic Resonance Spectroscopy (31P MRS) was used. The integral intensities of the resonant peaks and the phospholipid concentrations in PBMC and BMMC were analyzed.

[Factor VII deficiency--case report].

We describe the hypoproconvertinemia - a hereditary factor VII deficiency, diagnosed in 55-years-old female. Prolonged menstruations resulted in planned hysterectomy. Both her sons also have agent VII deficiency, however without any symptoms of hemorrhagic diathesis.

Lipid changes occuring in the course of hematological cancers.

The relationship between plasma lipid levels and mortality from cardiovascular diseases has been shown in many studies, but there has been far less investigation into their relationship to non-cardiovascular diseases. The aim of this study was to investigate the lipid profile of individuals with hematological malignancies and its relationship to disease activity. 238 patients were included in the study: 84 with acute leukemia, 62 with non-Hodgkin lymphoma, 35 with Hodgkin's lymphoma, 32 with multiple myeloma, and 25 with myeloproliferative syndrome.

Platelet-activating factor changes in phospholipid extracts from the plasma, peripheral blood mononuclear cells and bone marrow mononuclear cells of patients with acute leukemia--a 31P MRS in vitro study.

The aim of this investigation was to evaluate the changes in PAF concentrations in the plasma, PBMC and BMMC of patients with acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML). The plasma was from 23 healthy volunteers (HV) and 44 patients with AL (16 ALL, 28 AML). The PBMC were from 15 HV and 55 patients with AL (18 ALL, 37 AML), and the BMMC from 40 patients with AL (11 ALL, 29 AML).

Diffuse malignant lymphoma type B with optic chiasm infiltration, visual disturbances, hypopituitarism, hyperprolactinaemia and diabetes insipidus. Case report and literature review.

The case is reported of a 55-year-old man with diffuse malignant lymphoma type B associated with transient optic chiasm infiltration and visual disturbances but with persistent hypopituitarism, hyperprolactinaemia and diabetes insipidus. The patient was administered chemotherapy and radiotherapy. Repeated MR and CT scans showed optic chiasm infiltration, which disappeared in the course of the chemotherapy but then recurred, changed its appearance and finally disappeared again.

Non-Hodgkin's lymphoma as a rare manifestation of immune reconstitution disease in HIV-1 positive patients.

Combination antiretroviral therapy (cART) can improve immune system function through suppression of HIV-1 replication. However, paradoxical immune response may develop in some patients as a result of effective therapy followed by immune restoration. The phenomena is known as IRS, immune reconstitution syndrome/immune recovery syndrome.

[The biology of HDL lipoprotein and its antisclerotic activity].

Clinical and epidemiological studies showed an inverse relationship between the level of high-density lipoprotein (HDL) cholesterol and the development of atherosclerosis. This fact aroused more interest in HDLs and it was found that these lipoproteins have significance in malignant diseases. In this review the biochemical classification of plasma lipoproteins, the structure of HDL, and the structural characterization of HDL-apolipoproteins are presented.

[Glucose-6-phosphate dehydrogenase (G6PD) deficiency--a cause of anaemia in pregnant women].

Glucose-6-phosphate dehydrogenase (G6PD) is one of the most important cytoprotective enzymes for oxidative stress. The WHO classification of G6PD deficiency, based on enzyme activity and clinical significance, distinguishes five variants. Chronic haemolytic process is rare and the main factors causing haemolysis are: infections, substances derived from plants, drugs with high oxidation-reduction potential, stress, ketoacidosis in diabetes and surgery operations.

[Beta-Thalassemia in Poland. I. Mediterranean mutations in beta-thalassemia].

Unlabelled: The aim of the present investigation was to verify a common view that thalassemia in Poland is a very rare disease.

Material And Methods: 600 patients (270 male and 330 female) aged 2-85 years with microcytosis and no evidence of iron deficiency were examined for beta-thalassemia. Hemoglobin A2 and hemoglobin F and bilirubin were evaluated.

The significance of carboxy-terminal telopeptide of type I collagen (ICTP) and osteocalcin (OC) in assessment of bone disease in patients with multiple myeloma.

Bone disease occurring in multiple myeloma is usually evaluated using radiological methods. These methods, however, provide not much information about the dynamic process of bone resorption and formation. This study analysed levels of serum markers of bone turnover (ICTP and OC), reflecting function of osteoclasts and osteoblasts.

31P MRS analysis of the phospholipid composition of normal human peripheral blood mononuclear cells (PBMC).

The aim of this investigation was to characterize the phospholipid composition of normal human blood mononuclear cells using 31P NMR spectroscopy. Mononuclear cells of peripheral blood were obtained from 10 volunteers. Phospholipid extracts were prepared from 60x10(6) cells according to modified Folch's method.

Application of 31P MRS to the analysis of phospholipid changes in plasma of patients with acute leukemia.

The aim of the experiment was to evaluate the changes of phospholipid concentrations in patients (n=30) with acute leukemia compared with reference group of healthy volunteers (n=21). The analysis focused on the following phospholipids (PL) collected from plasma: phosphatidylcholine (PC), plasmalogen of phosphatidylcholine (CPLAS), lysophosphatidylcholine (LPC), sphingomyelin (SM), phosphatidylethanolamine (PE), and phosphatidylinositol (PI). Phospholipid extracts were obtained by Folch's method from 4 ml of plasma.