vitamin-k-semiquinone-radical and Hemoglobinuria--Paroxysmal

Antiphospholipid syndrome (APS), heparin-induced thrombocytopenia, and paroxysmal nocturnal hemoglobinuria are 3 acquired thrombophilias that carry a high risk of venous and arterial thromboembolism. Management of these conditions has largely included anticoagulation with a vitamin K antagonist after an initial period of a parenteral anticoagulant, for as long as the thrombotic risk is still present. The available evidence for the use of direct oral anticoagulants (DOACs) is limited and primarily consists of case series and cohort studies, which are summarized in this chapter. Randomized trials evaluating DOACs in patients with APS are reviewed. Further research is needed prior to widely adopting DOACs for use in these high-risk acquired thrombophilias; however, there may be selected low-risk subgroups where DOAC use is possible after careful consideration and patient discussion.

Topics: Administration, Oral; Anticoagulants; Antiphospholipid Syndrome; Disease Management; Hemoglobinuria, Paroxysmal; Heparin; Humans; Thrombocytopenia; Thrombophilia; Thrombosis; Vitamin K

Antiphospholipid syndrome (APS), heparin-induced thrombocytopenia, and paroxysmal nocturnal hemoglobinuria are 3 acquired thrombophilias that carry a high risk of venous and arterial thromboembolism. Management of these conditions has largely included anticoagulation with a vitamin K antagonist after an initial period of a parenteral anticoagulant, for as long as the thrombotic risk is still present. The available evidence for the use of direct oral anticoagulants (DOACs) is limited and primarily consists of case series and cohort studies, which are summarized in this chapter. Randomized trials evaluating DOACs in patients with APS are reviewed. Further research is needed prior to widely adopting DOACs for use in these high-risk acquired thrombophilias; however, there may be selected low-risk subgroups where DOAC use is possible after careful consideration and patient discussion.

Topics: Administration, Oral; Anticoagulants; Antiphospholipid Syndrome; Hemoglobinuria, Paroxysmal; Heparin; Humans; Patient Education as Topic; Risk Factors; Thrombocytopenia; Thrombophilia; Vitamin K

Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired, life-threatening haematological disorder. It is characterised by complement induced haemolytic anaemia, thrombosis and impaired bone marrow function. Thrombosis most commonly occurs in the hepatic, portal, superior mesenteric and cerebral veins. A22-year female, previously diagnosed with severe aplastic anaemia treated with anti-lymphocyte globulin (ALG) and cyclosporine, had become transfusion independent for more than 10 years. She presented with abdominal pain and vomiting, initially diagnosed with portal and superior mesenteric vein thrombosis. Immunophenotyping by flow cytometry revealed a diagnosis of paroxysmal nocturnal haemoglobinuria type III. She was treated with vitmamin K anatagonist and platelet transfusion.

Topics: Adult; Female; Flow Cytometry; Hemoglobinuria, Paroxysmal; Humans; Immunophenotyping; Mesenteric Veins; Platelet Transfusion; Thrombosis; Treatment Outcome; Vitamin K; Vitamins

Topics: Amoxicillin; Anticoagulants; Blood Coagulation Tests; Citalopram; Contraindications; Drug Therapy, Combination; Enoxaparin; Hemoglobinuria, Paroxysmal; Humans; Male; Penicillanic Acid; Piperacillin; Piperacillin, Tazobactam Drug Combination; Plasma; Platelet Transfusion; Prednisolone; Prednisone; Protein C Deficiency; Purpura Fulminans; Remission Induction; Virus Diseases; Vitamin K; Warfarin; Young Adult

Budd-Chiari syndrome with or without portal thrombosis occurring during paroxysmal noctural hemoglobinuria is a complication with poor prognosis. We report the case of a 17-year-old woman with a double portal and hepatic venous thrombosis revealing a paroxysmal noctural hemoglobinuria and regressive with heparin. Our case suggests that the early diagnosis of the thrombosis with ultrasonography and Doppler, and rapidly initiated anticoagulant treatment may improve the prognosis of this disease.

Topics: 4-Hydroxycoumarins; Adolescent; Anticoagulants; Budd-Chiari Syndrome; Female; Hemoglobinuria, Paroxysmal; Heparin; Humans; Indenes; Portal Vein; Thrombosis; Vitamin K