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Introduction: Polycythemia vera (PV) is a rare myeloproliferative disease associated with an increased prevalence of hypertension and increased risk of cardiovascular complications. However, the precise mechanisms leading to the elevation of blood pressure (BP) and secondary target organ damage remain poorly understood., Objectives: The study aimed to evaluate BP profile, assess the activity of the sympathetic nervous system and the renin‑angiotensin system, and provide a comprehensive assessment of subclinical target organ damage in patients with PV., Patients and Methods: Twenty consecutive patients with newly diagnosed PV and 20 control subjects were included. The following were assessed: BP, levels of catecholamines, urinary and plasma O‑methylated catecholamine metabolites, concentrations of aldosterone and renin. We also assessed microneurography sympathetic nervous system activity (MSNA) and baroreflex control of heart rate as well as subclinical target organ damage., Results: At similar levels of BP, BP variability was decreased in the PV group (mean [SD] 24‑hour systolic BP, 9 [3] vs 12 [3] mm Hg; P = 0.003). Patients with PV had lower norepinephrine excretion (mean [SD], 16.54 [6.32] vs 25.46 [12.88] μg/d; P = 0.03) as well as decreased MSNA as assessed by microneurography compared with controls (mean [SD] MSNA, 30.7 [8.7] bursts/min vs 38.7 [5.4] bursts/min; P = 0.007 and MSNA 51.8 [11] bursts/100 beats vs 61.1 [11.3] bursts/100 heart beats; P = 0.04). Baroreflex control of HR was unaltered in the PV group. Increased hemoglobin levels and red blood cell count correlated with decreased retinal capillary flow in patients with PV., Conclusions: Patients with PV, characterized by high hemoglobin concentrations and hematocrit levels had lower sympathetic nervous activity and decreased BP variability as compared with controls. There was no relationship between hemoglobin plasma concentration, hematocrit level, and target organ damage.

Direct oral anticoagulants (DOACs) such as apixaban, dabigatran, edoxaban, and rivaroxaban are mainly used in the prevention of thromboembolic complications in patients with atrial fibrillation (AF) and in the treatment of venous thromboembolism. As compared with vitamin K antagonists (VKAs), they are characterized by at least similar efficacy and better safety profiles, especially with respect to intracranial hemorrhages. Moreover, they are more convenient therapeutic agents. The 2016 European Society of Cardiology guidelines clearly favor DOACs over VKAs in patients with AF. However, DOAC therapy is also associated with the risk of bleeding complications. The aim of this review was to provide recommendations for the management of bleeding complications during DOAC therapy in the Polish setting. The recommendations were based on the most important documents concerning this issue and were developed by representatives of different medical specialties. Experience in managing cases of bleeding on DOAC therapy is still limited. Therefore, we hope that this publication will be helpful in everyday clinical practice and that it will be useful for developing in‑hospital recommendations for the management of patients with DOAC‑related bleeding.

Dabigatran, a direct thrombin inhibitor and 2 factor Xa inhibitors, rivaroxaban and apixaban, are target-specific oral anticoagulants (TSOACs) approved for prevention of stroke or systemic embolism in patients with nonvalvular atrial fibrillation (AF). Published data suggest that all 3 agents are at least as efficacious as dose‑adjusted warfarin in stroke prevention. Because of their greater specificity, rapid onset of action, and predictable pharmacokinetics, TSOACs have some advantages over vitamin K antagonists, which facilitates their use in clinical practice. The current review addresses the practical questions relating to the use of TSOACs in AF patients based on the available data and personal experience. We discuss topics such as patient selection, renal impairment, drug interactions, switching between anticoagulants, laboratory monitoring, and the risk of bleeding along with its management. We will focus on the aspects of the optimization of treatment with TSOACs in stroke prevention. The understanding of these practical issues by clinicians and patients is of key importance for the safe and effective use of TSOACs in everyday practice.

Antiphospholipid syndrome (APS) is an autoimmune disease with clinical manifestations of arterial and venous thrombosis, concomitant fetal loss and the presence of antiphospholipid antibodies (APLA). This report focuses on the challenges of optimal treatment involving plasma exchange and intravenous human immunoglobulin infusions that is administered in patients with catastrophic APS (CAPS). CAPS is a rare variant of APS defined as acute failure of at least three tissues, organs or systems caused predominantly by small vessel thrombosis confirmed by histopathologic evidence. CAPS develops rapidly and leads to death in 50% of cases. We present the case of a 39-year-old male patient with APS with worsening renal function. Positive lupus anticoagulant, markedly high concentrations of anticardiolipin and anti-beta 2-glikoprotein I antibodies have been observed. According to the criteria introduced by Asherson, a catastrophic form of APS was diagnosed and the patient had been treated with low-molecular-weight heparin, glucocorticosteroids, and plasmapheresis. In order to maintain clinical improvement, the patient was given human immunoglobulins i.v. (1 g/kg body weight). After the procedure, gradual clinical improvement was observed and renal function remained stable (serum creatinine level of 1.5 mg/dl).

Advances in hemophilia management in the 20th century enabled effective and early treatment of joint and other bleeds typical of this disease, also in a home setting. Prophylaxis became available as the optimal approach to prevent hemophilic arthropathy and improve patients' quality of life. To increase treatment safety, lyophilized plasma-derived factor VIII and IX concentrates were subjected to numerous procedures designed to decrease the risk of transmission of known and unknown pathogens. During the following years, recombinant factor VIII and factor IX preparations were developed to completely eliminate the risk. Recombinant factor concentrates were extensively studied in terms of their therapeutic properties, safety, and immunogenicity. This article reviews the current knowledge on efficacy and safety of recombinant factors VIII and IX.

Acquired hemophilia is a severe bleeding diathesis that affects both males and females. It is caused by suddenly appearing autoantibodies that interfere with coagulation factor VIII activity. This disorder is characterized by spontaneous and post-traumatic subcutaneous bleeds and massive mucosal hemorrhages. We report in the current article a case of acute renal failure and bleeding from the urinary tract caused by idiopathic acquired hemophilia in a 54-year-old woman. Hemostatic tests indicated prolonged activated partial thromboplastin time (APTT) to 107.8 sec (norm 26-36 sec), normal value of the prothrombin index which was 82% (norm 70-130%), increased fibrinogen concentration to 583 mg/dl (normal value 200-400 mg/dl), the bleeding time was 5 min and 20 s (norm < 10 min) and the platelet count was 366 x 10(9)/l (norm 130-400 x10(9)/l). The autoantibody against factor VIII in a titer of 121 Bethesda Units/ml (BU/ml) and decreased factor VIII activity to 2% (norm 50-150%) with normal plasma concentration of factor IX. Activated (FEIBA, Baxter) and nonactivated prothrombin complex concentrates (factor IX concentrate) have been used in the treatment of bleeding episode. Immunosuppressive treatment with the combination of oral prednisone 60 mg/24h and cyclophosphamide 150 mg/24h was administered in order to remove the factor VIII inhibitor. Reduction of the factor VIII inhibitor titer to 38 BU/ml and increase of factor VIII activity to 4% was initially achieved. This treatment has been continued for two years and led to normalization of hemostatic parameters (APTT 26 sec, factor VIII activity 108%) which means a total removal of factor VIII inhibitor.

Acquired haemophilia (AH) is a severe bleeding diathesis that affects both males and females. It is caused by suddenly appearing autoantibodies that interfere with coagulation factor VIII (FVIII) activity. Although some conditions such as autoimmune diseases, cancer and puerperium seem likely to induce AH, in more than half of the observed cases autoantibodies to FVIII are idiopathic. The clinical picture is characterized by spontaneous and post-traumatic subcutaneous bleeds as well as massive mucosal membrane hemorrhages (from the genitourinary and gastrointestinal tracts). Typical abnormalities in AH are prolonged activated partial thromboplastin time and normal results of the other haemostatic tests (platelet count, prothrombin and thrombin times, fibrinogen concentration). Acquired haemophilia is definitely confirmed by quantification of FVIII neutralizing antibodies. Bleeds are usually treated with activated prothrombin complex concentrates and activated recombinant factor VII. In most patients with AH, the use of immunosuppressive agents results in autoantibody elimination and restoration of normal FVIII plasma activity.

The aim of the present study was to describe the orthopaedic status of patients with severe haemophilia, and to relate this status to the type of replacement therapy received by patients prior to the study. Ninety two haemophiliacs with median age 26 were included. Six joints--knees, elbows and ankles were evaluated clinically using the Gilbert scale. The evaluation included physical status (0-12 points/joint) and pain score (0-3 points/joint). X-ray examinations were evaluated according to the Pettersson scale (0-13 points/joint). On all scales, normality was represented by 0 score. Knees were the most affected joints. Eighty four patients (91.3%) reported pain. Only one patient scored 0 on the Gilbert scale, and another on the Pettersson scale. Thirty seven percent of patients used orthopaedic equipment occasionally or constantly. Twenty five percent of patients had a history of orthopaedic surgery. Thirty eight percent were unemployed and received some form of social subvention. On demand treatment was applied. None of the patients received primary prophylaxis. The mean consuption of clotting factor concentrates was 68,054 IU per patient during the 12 months period prior to the current study. The results of this study indicate that vast majority of severe haemophilia patients in Poland above 20 are affected by haemophilic arthropathy. This disabling complication of severe haemophilia can be prevented only by introducing primary prophylaxis from the first years of life.

The aim of the study was to analyze the data on 3224 patients with inherited blood coagulation disorders registered in Poland till December 1st, 2003. In 2269 registered hemophilia patients, 1953 were hemophilia A, and 316 were hemophilia B. Hemophilia A occurs in 1512 families, and hemophilia B in 240 families. Severe hemophilia constitutes the majority of hemophilia A and B cases (59.7% and 56.6% respectively). About 50% of the hemophiliacs have no history of bleeding diathesis in the family. The mean age of hemophilia A patients is 30.9 years, and that of hemophilia B patients--29.2 years. Prevalence of hemophilia in Poland is approximately 1:12 300 inhabitants. Hemophilia A has been diagnosed in 60.6% of all patients with inherited blood coagulation disorders registered in Poland, von Willebrand disease-- in 21.9%, hemophilia B-- in 9.8% and factor VII deficiency-- in 3.3%.

The aim of the following study was to determine the prevalence of lupus anticoagulant (LA) and anticardiolipin antibodies (ACL) in patients with a history of venous thromboembolism (VTE). The patient group comprised 218 subjects with VTE before the age of 45, recurrent VTE or thrombosis in an unusual site. The control group consisted of 218 age, and sex-matched healthy individuals. LA and/or ACL were detected in 19 among 218 patients (8.7%). Lupus anticoagulant was found in 17 patients with VTE and in none out of 218 controls. The odds ratio for having venous thromboembolism was 14.1 (95% CI: 1.8-108.8) for patients with LA. Lupus anticoagulant is significantly associated with VTE. The prevalence of anticardiolipin was similar in patients and in controls. The results of our study indicate that anticardiolipin antibodies are not associated with venous thromboembolism.

The G20210A mutation of the prothrombin (PT) gene has recently been identified as a risk factor for venous thromboembolism (VTE). This mutation was shown to be present mainly among Caucasian populations, with a higher frequency in southern than in northern Europe. The aim of our study was to determine the prevalence of the PT 20210A allele in the Polish general population and in patients with a history of venous thrombosis. The patient group comprised 323 subjects with VTE before the age of 45, recurrent VTE or thrombosis in an unusual site. The control group consisted of 399 healthy individuals. Heterozygosity for the PT 20210A allele was found in 21 (6.5%) patients and 7 (1.8%) controls. In 7 (33.3%) of the 21 heterozygous patients the PT 20210A allele was associated with the factor V Leiden mutation, in 1--with the homozygous C677T mutation of the methylenetetrahydrofolate reductase (MTHFR), and in 1--with lupus anticoagulant. Our results indicate that the presence of the 20210A allele is a mild risk factor for venous thrombosis if not associated with other thrombophilic defect (odds ratio 2.2; 95% CI: 0.8-5.5). The risk is greater in double heterozygous carriers of the PT 20210A allele and factor V Leiden mutation.