warfarin and Hemoglobinuria--Paroxysmal

A 28-year-old man was hospitalized with nausea, vomiting, abdominal pain and low-grade fever. He had a 6-month history of paroxysmal nocturnal haemoglobinuria (PNH), and laboratory data showed anaemia and liver dysfunction. An abdominal ultrasonography showed ascites and portal vein thrombosis. After receiving antithrombotic treatment, the portal vein thrombosis did not extend. Portal vein thrombosis is very rare but should be considered when we encounter liver dysfunction associated with PNH as well as hepatic vein thrombosis. Ultrasonography is very useful in detecting portal vein thrombosis and facilitating early diagnosis. Warfarin is very effective in preventing exacerbation of portal vein thrombosis in PNH.

Topics: Adult; Anticoagulants; Hemoglobinuria, Paroxysmal; Humans; Liver Function Tests; Male; Portal Vein; Tomography, X-Ray Computed; Ultrasonography; Venous Thrombosis; Warfarin

Topics: Androgens; Anemia, Hemolytic, Autoimmune; Antigen-Antibody Reactions; Antigens; Autoantibodies; Blood Transfusion; Cold Temperature; Complement System Proteins; Dextrans; Hemagglutination; Hemoglobinuria, Paroxysmal; Hemolysis; Humans; Immunoglobulin G; Immunoglobulin M; Immunosuppression Therapy; Prednisone; Splenectomy; Warfarin

Topics: Abdominal Pain; Adult; Anemia, Hemolytic; Antibodies, Monoclonal, Humanized; Anticoagulants; Budd-Chiari Syndrome; Complement Inactivating Agents; Drug Therapy, Combination; Ferritins; Hemoglobin A; Hemoglobinuria, Paroxysmal; Hemolysis; Hepatomegaly; Humans; Male; Tomography, X-Ray Computed; Treatment Outcome; Warfarin

Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired clonal haematological disease characterized by complement-mediated haemolysis, bone marrow failure and venous thrombosis. Anticomplement therapy eculizumab improves survival and reduces complications. Severe acute respiratory distress syndrome corona virus 2 (SARS-CoV-2) disease 2019 (COVID-19) is associated with high incidence of both venous and arterial thrombosis in hospitalized patients with pneumonia. Deep venous thrombosis (DVT) as the presenting symptom of COVID-19 is a rare event. We describe a well-controlled PNH patient on eculizumab for more than 5 years who presented with DVT, while on warfarin, as the first sign of COVID-19. To our knowledge, this is the first described case of DVT in a PNH patient with COVID-19.

Topics: Adult; Antibodies, Monoclonal, Humanized; Anticoagulants; Complement Inactivating Agents; COVID-19; Hemoglobinuria, Paroxysmal; Humans; Male; SARS-CoV-2; Venous Thrombosis; Warfarin

Topics: Anemia, Hemolytic; Antibodies, Monoclonal, Humanized; Diagnosis, Differential; Fatigue; Female; Headache; Hematologic Agents; Hemoglobinuria, Paroxysmal; Hemolysis; Humans; Middle Aged; Patient Care Management; Thrombosis; Warfarin

Topics: Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Antilymphocyte Serum; Cerebrovascular Disorders; Child; Child, Preschool; Cyclosporine; Erythrocyte Transfusion; Female; Hemoglobinuria, Paroxysmal; Hemolysis; Heparin; Humans; Infant; Infant, Newborn; L-Lactate Dehydrogenase; Male; Middle Aged; Myocardial Infarction; Neutropenia; Neutrophils; Registries; Retrospective Studies; Thromboembolism; Warfarin

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

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired genetic disorder of the bone marrow that produces intravascular hemolysis, proclivity to venous thrombosis, and hematopoietic failure. Mutation in the PIG-A gene in a stem cell aborts synthesis of glycosyl-phosphoinositol (GPI) anchors and therefore expression of all GPI-anchored proteins on the surface of progeny erythrocytes, leukocytes, and platelets. The hemolytic anemia of PNH is well understood, and erythrocyte susceptibility to complement may be treated with anti-C5a monoclonal antibody. The pathophysiology of PNH cell clonal expansion and its association with immune-mediated marrow failure are not understood, but PNH/aplasia responds to immunosuppressive regimens such as antithymocyte globulin and cyclosporine. The mechanism of thrombosis in PNH is also obscure, but frequently fatal clotting episodes may be prevented by Coumadin (Bristol-Myers Squibb Pharma Co., Wilmington, DE) prophylaxis.

Topics: Antibodies, Monoclonal; Bone Marrow Diseases; Hemoglobinuria, Paroxysmal; Humans; Membrane Proteins; Mutation; Stem Cell Transplantation; Warfarin

Topics: Adult; Ascites; Budd-Chiari Syndrome; Factor V; Hemoglobinuria; Hemoglobinuria, Paroxysmal; Heterozygote; Humans; International Normalized Ratio; Male; Methylenetetrahydrofolate Dehydrogenase (NAD+); Mutation; Prothrombin; Spleen; Thrombophilia; Time Factors; Tomography, X-Ray Computed; Warfarin

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which venous thrombosis is the most common cause of death. Here we address the risk factors for thrombosis and the role of warfarin prophylaxis in PNH. The median follow-up of 163 PNH patients was 6 years (range, 0.2-38 years). Of the patients, 29 suffered thromboses, with a 10-year incidence of 23%. There were 9 patients who presented with thrombosis, and in the remainder the median time to thrombosis was 4.75 years (range, 3 months-15 years). The 10-year risk of thrombosis in patients with large PNH clones (PNH granulocytes > 50%) was 44% compared with 5.8% with small clones (P <.01). Patients with large PNH clones and no contraindication to anticoagulation were offered warfarin. There were no thromboses in the 39 patients who received primary prophylaxis. In comparison, 56 patients with large clones and not taking warfarin had a 10-year thrombosis rate of 36.5% (P =.01). There were 2 serious hemorrhages in more than 100 patient-years of warfarin therapy. Large PNH granulocyte clones are predictive of venous thrombosis, although the exact cut-off for clone size is still to be determined. Primary prophylaxis with warfarin in PNH prevents thrombosis with acceptable risks.

Topics: Clone Cells; Follow-Up Studies; Hemoglobinuria, Paroxysmal; Humans; Incidence; Retrospective Studies; Risk Factors; Thrombosis; Treatment Outcome; Warfarin

A 44-year-old Japanese man having aplastic anemia (AA)-paroxyamal nocturnal hemoglobinuria (PNH) syndrome was referred to our hospital because of purpuras due to thrombocytopenia in July 1994. He suffered from pneumonia after admission, complicated with cerebral, splenic, and left renal infarction. Pulmonary infaction was also confirmed by perfusion lung scan. He had a plasma plasminogen (PLG) functional activity of 54.4% with a normal level of PLG antigen. The gel isoelectrofocusing pattern of the plasminogen derived from the patient showed 10 normal bands and 10 additional doublet bands with slightly higher isoelectric points than the normal components. Abnormal PLG is converted by urokinase to an inactive two-chain plasmin molecule. These findings were similar to those of a case with dysplasminogenemia (PLG Tochigi) reported by Aoki et al. He was given warfarin for the prevention of thrombosis in December 1994. As of October 1995, these was no recurrence of thrombosis. The cause of thrombosis in the present case have been the association with PNH, predisposition to PLG Tochigi, or the complication of pneumonia. This is the first report of AA/PNH syndrome associated with dysplasminogenemia.

Topics: Adult; Anemia, Aplastic; Anticoagulants; Hemoglobinuria, Paroxysmal; Humans; Male; Plasminogen; Pneumonia; Syndrome; Thrombosis; Warfarin

Hepatic venous thrombosis (HVT) should be recognized as a distinct and highly lethal thrombotic complication of paroxysmal nocturnal hemoglobinuria. In a patient with fulminant onset prompt recognition of a triad of clinical, laboratory and liver scan findings facilitated early, aggressive and prolonged heparinization followed by coumadin maintenance, all with good results. Additionally a case of asymptomatic, smoldering HVT was unearthed by liver scan survey and confirmed by hepatic venogram; the patient was started on a regimen of Coumadin (crystalline sodium warfarin, Endo).

Topics: Budd-Chiari Syndrome; Emergencies; Hemoglobinuria, Paroxysmal; Heparin; Humans; Male; Middle Aged; Warfarin

Topics: Adult; Animals; Antibodies; Budd-Chiari Syndrome; Complement Fixation Tests; Complement System Proteins; Female; Guinea Pigs; Hemoglobinuria, Paroxysmal; Hemolysis; Humans; Inulin; Male; Middle Aged; Prednisolone; Rabbits; Sheep; Snakes; Sucrose; Venoms; Warfarin

Topics: Adult; Anticoagulants; Drug Tolerance; Hemoglobinuria, Paroxysmal; Humans; Male; Oxymetholone; Phenindione; Warfarin