Adherence to prophylactic treatment in adult patients with severe hemophilia in Poland.

Introduction: Patient adherence to a prophylactic regimen is important for optimal benefit of hemophilia treatment. Despite a growing number of adults with hemophilia in Poland receiving secondary prophylaxis, data on adherence to the regimen are limited.

Objectives: The aim of the study was to assess adherence to secondary prophylaxis in Polish adults with severe hemophilia.

Genetic and clinical characterization of congenital fibrinogen disorders in Polish patients: Identification of three novel fibrinogen gamma chain mutations.

Introduction: Congenital fibrinogen disorders are poorly explored in Slavic populations. The aim of this study was to characterize the genetic background and clinical manifestations of fibrinogen disorders in the Polish case series.

Materials And Methods: In 27 unrelated patients (mean [SD] age, 30.

Mechanical methods of venous thromboembolism prevention: from guidelines to clinical practice.

The paper discusses guidelines for the prevention of venous thromboembolism (VTE), with particular emphasis on the use of mechanical methods. Mechanical prophylaxis of VTE does not involve the risk of bleeding, which may be life‑threatening. Mechanical methods are particularly recommended in patients at high risk of bleeding while on pharmacological thromboprophylaxis.

Genetic characterization of antithrombin, protein C, and protein S deficiencies in Polish patients.

INTRODUCTION    Inherited deficiencies of natural anticoagulants such as antithrombin (AT; gene: SERPINC1), protein C (PC; PROC), and protein S (PS; PROS1), with the prevalence in the general European population of 0.02% to 0.17%, 0.

Efficacy, safety and pharmacokinetic profiles of a plasma-derived VWF/FVIII concentrate (VONCENTO®) in subjects with haemophilia A (SWIFT-HA study).

Introduction: VONCENTO® (CSL Behring) is a plasma-derived, high-concentration, low-volume, high-purity concentrate,which contains a high level of von Willebrand factor (VWF) high-molecular-weight multimers and aVWF/factor VIII (FVIII) ratio of ~2.4:1, similar to Haemate® P (CSL Behring).

Methods: The pharmacokinetic, efficacy and safety profiles of VONCENTO® were investigated in this multicentre,double-blind, randomised study.

[Hemoptysis during concomitant treatment with rivaroxaban and amiodarone in a patient with a history of pulmonary disease].

Unlabelled: Rivaroxaban, a selective inhibitor of active factor X, is metabolized by cytochrom P450 3A4 (CYP3A4) and is a substrate for transporter protein--P-glycoprotein (P-gp). Amiodarone, an antiarrhytmic agent, is classified as moderate CYP3A4 and P-gp inhibitor.

A Case Report: A 75-year-old male, who underwent lobectomy for bronchiectasis many years ago, is presented.

Intracranial bleedings in patients on long-term anticoagulant treatment: Benefits from oral thrombin and factor Xa inhibitors in clinical practice.

Dabigatran, a direct thrombin inhibitor and activated factor X inhibitors, rivaroxaban and apixaban, used in the prevention of stroke or systemic embolism in patients with nonvalvular atrial fibrillation (AF), have several advantages over vitamin K antagonists (VKAs). The non-vitamin K oral anticoagulants (NOACs) have been shown to reduce the risk of intracranial bleedings by 50%. The current review summarizes the available data on the epidemiology, mechanisms and treatment of intracranial bleedings observed on oral anticoagulation with the focus on the specificity of NOACs in this context.

Long-term results of the Polish Adult Leukemia Group PALG-CLL2 phase III randomized study comparing cladribine-based combinations in chronic lymphocytic leukemia.

Long-term outcomes following newer therapies for chronic lymphocytic leukemia (CLL) have rarely been reported. This article presents the results of the final analysis of the Polish Adult Leukemia Group PALG-CLL2 study performed 10 years from final patient enrollment. With the extended follow-up time, it was found that cladribine (2-CdA)-based combinations CMC (2-CdA, cyclophosphamide, mitoxantrone) and CC (2-CdA, cyclophosphamide) administered as first-line treatment of progressive CLL resulted in significantly longer progression-free survival, but similar overall survival compared to 2-CdA monotherapy.

Immune thrombocytopenia in patients with chronic lymphocytic leukemia treated with cladribine-based regiments or chlorambucil--follow-up of PALG-CLL randomized trials.

Objectives: The relationship between treatments of chronic lymphocytic leukemia (CLL) with cladribine (2-CdA) or chlorambucil and immune thrombocytopenia (IT) has not been yet determined.

Methods: The records of 777 patients in two randomized Polish Adult Leukemia Group (PALG)-CLL programs treated with these agents were retrospectively analyzed.

Results: Immune thrombocytopenia occurred in 55 of 777 (7.

[Polish guidelines for the prevention and treatment of venous thromboembolism. 2012 update].

The overall objective of the Polish guidelines for the prevention and treatment of venous thromboembolism is to increase patient benefit and safety by appropriate prevention and treatment of deep vein thrombosis and pulmonary embolism as well as proper management of the complications associated with antithrombotic and thrombolytic therapy. These guidelines apply to adult trauma, cancer, surgical, and medical patients as well as those at increased risk of venous thromboembolism. Specific recommendations have been formulated for pregnant women, patients requiring surgery while receiving long-term oral anticoagulant treatment, and patients undergoing regional anesthesia and/or analgesia.

Therapeutic problems in elderly patients with hemophilia.

Since the introduction of clotting factor concentrates, the life expectancy of patients with hemophilia has increased from 40 years in the 1960s to 60 or even 70 years today. In Poland, almost all elderly patients with hemophilia have arthropathy, the majority are infected with hepatitis C virus (HCV), and some even with hepatitis B or human immunodeficiency virus. Liver cirrhosis associated with HCV infection develops within 15 to 20 years in 20% to 30% of these patients.

Cladribine, but not fludarabine, added to daunorubicin and cytarabine during induction prolongs survival of patients with acute myeloid leukemia: a multicenter, randomized phase III study.

Purpose: The goal of this study was to evaluate whether the addition of a purine analog, cladribine or fludarabine, to the standard induction regimen affects the outcome of adult patients with acute myeloid leukemia (AML).

Patients And Methods: A cohort of 652 untreated AML patients with median age 47 years (range, 17 to 60 years) were randomly assigned to receive one of three induction regimens: DA (daunorubicin plus cytarabine), DAC (DA plus cladribine), or DAF (DA plus fludarabine). Postremission treatment was the same for all arms.

[Severe pulmonary embolism in a young marijuana smoker].

It is a popular belief, that marijuana smoking is not harmful to health. Some publications, however, suggest its possible association with mental, respiratory and cardiovascular complications, but not with venous thromboembolism. The authors describe a case of severe pulmonary embolism in a mildly obese, 22 year-old marijuana and tobacco smoker.

[Depression, its therapy and venous thromboembolism: three cases of pulmonary embolism].

The associations between depression and coronary heart disease, especially via platelet hyperactivity, have been widely described. The relationships between depression and venous thromboembolism are less clear. We present three cases of pulmonary embolism (PE) in patients with previously diagnosed depression and discuss possible, depression-related prothrombotic factors, including the impact of psychotropic drugs.

[Venous thromboembolism triggered by spinning in a young woman with thrombophilia].

Although regular sports activities decrease the risk of venous thromboembolism (VTE), VTE cases have been observed among professional and amateur athletes practicing various disciplines. The authors describe a case of a 25-year-old-woman in whom calf pain, as popliteal vein thrombosis manifestation--preceding pulmonary embolism, occurred after she took up spinning, a popular form of indoor cycling. There was no history of leg injury.

["eThrombosis" - venous thromboembolism provoked by prolonged sitting position by a computer. A case series report of six patients].

Immobility due to sitting position during traveling is a well-known risk factor for venous thromboembolism (VTE). Beasley et al. (2003) first proposed the name "eThrombosis" for a case in which VTE was related to prolonged seated immobility at computer.

Clinical assessment of Optivate®, a high-purity concentrate of factor VIII with von Willebrand factor, in the management of patients with haemophilia A.

Factor VIII (FVIII) concentrates have revolutionized the treatment of patients with haemophilia A. Concerns over the transmission of viral infections through these products have been addressed through stringent, donor-screening procedures and robust antiviral manufacturing steps. Bio Products Laboratory has developed a high-purity FVIII product with von Willebrand factor, Optivate(®).

[Pulmonary embolism in a 30 year-old man with chronic hepatitis C during therapy with pegylated interferon-α and ribavirin].

The authors described a case of a 30 year-old man with chronic hepatitis C (G1,S1) complicated by pulmonary embolism (PE), preceded by symptoms of respiratory system infection, which occurred after 6 months of combination therapy with pegylated interferon-α and ribavirin. Right sided nonbacterial thrombotic endocarditis as a source of segmental/subsegmental PE was found. Hemostatic work up showed increased activated protein C resistance, elevated factor VIII activity and fibrinogen concentration as well as hyperhomocysteinaemia (all these disturbances returned to normal 3-6 months later).

Pharmacokinetics of Optivate(®), a high-purity concentrate of factor VIII with von Willebrand factor, in patients with severe haemophilia A.

Optivate(®) is a high purity factor VIII/von Willebrand factor (FVIII/VWF) concentrate, which is manufactured using two antiviral processes: solvent/detergent and terminal dry heating (80 °C for 72 h). A multicentre, non-randomized open-label study in 15 patients was conducted to test the pharmacokinetics (PK) of Optivate(®). PK variables were analysed for the patients' prior FVIII product (PK1), their first dose of Optivate(®) (PK2) and at 3 months therapy (PK3).

Registry of inherited bleeding disorders in Poland--current status and potential role of the HemoRec database.

We present data collected in HemoRec, an Internet-based platform implemented in 2006 in 15 haemophilia treatment centres in Poland and compare them with the national registry of inherited bleeding disorders established since 1991 at the Institute of Haematology and Blood Transfusion in Warsaw. We also analyse the current status of haemophilia treatment in Poland as well as future perspectives. Data on 1102 patients registered in HemoRec were analysed and compared with 4294 patients in the national registry (status as at 17.