To determine laboratory evidence suggesting immunological abnormalities in persons with hemophilia, we evaluated the immunological status of 75 Korean hemophiliacs, seronegative for human immunodeficiency virus (HIV) antibodies, who have been treated only with Korean factor VIII concentrates. From this study, it was shown that Korean hemophiliacs had decreased CD4 levels, increased CD8 levels, and decreased CD4:CD8 ratios. Diminished lymphocyte response to the mitogens, phytohemagglutinin and concanavalin A, and decreased natural killer cell activity were observed in the hemophiliacs. In addition, production of interleukin-II in the hemophiliacs was lower than in the healthy controls. The percentage of B lymphocytes was significantly reduced but the serum levels of immunoglobulin (Ig) G were elevated. However, the serum Ig A and Ig M levels were normal. This study demonstrated a high frequency of immunological abnormalities in HIV antibody negative Korean hemophiliacs treated only with domestic factor VIII concentrates.
Adolescent ; Adult ; Child ; Factor VIII/therapeutic use ; HIV Seropositivity/immunology ; Hemophilia A/drug therapy/*immunology ; Human ; Korea ; Lymphocyte Activation ; Lymphocytes/immunology ; Male ; Middle Age ; Support, Non-U.S. Gov't

Acquired hemophilia A (AHA) is a rare coagulopathy caused by autoantibodies to coagulation factor VIII (FVIII). Most patients with AHA have been previously healthy; however, a variety of morbidities have been associated with the condition including pregnancy. A 40-yr-old woman visited our institution with extensive hematoma on the right hip area. Her medical history revealed no personal or familial history of bleeding diathesis. Her coagulation tests showed markedly prolonged aPTT (117 sec), markedly decreased level of FVIII activity (0.4%) and high-titer FVIII inhibitor (77 BU). Collectively, she was diagnosed as having postpartum AHA and was treated with bypassing agents and corticosteroids. Her aPTT was normalized on the 174th postpartum day and FVIII inhibitor showed negative conversion on the 224th postpartum day. This is the first case of postpartum AHA with high-titer FVIII inhibitor in Korea. Timely diagnosis and management can reduce morbidity and mortality of this potentially life-threatening condition.
Adrenal Cortex Hormones/therapeutic use ; Adult ; Autoantibodies/blood ; Blood Coagulation Factors/therapeutic use ; Factor VIII/immunology ; Factor VIIa/therapeutic use ; Female ; Hematoma/diagnosis ; Hemophilia A/*diagnosis/therapy ; Humans ; Partial Thromboplastin Time ; Postpartum Period ; Pregnancy ; Recombinant Proteins/therapeutic use ; Republic of Korea

Blood Coagulation Factor Inhibitors ; antagonists & inhibitors ; blood ; Factor VIII ; administration & dosage ; antagonists & inhibitors ; immunology ; Hemophilia A ; drug therapy ; genetics ; immunology ; Humans ; Immune Tolerance ; Isoantibodies ; blood ; immunology ; Recombinant Proteins ; adverse effects ; immunology ; therapeutic use ; Risk Factors ; Time Factors

PURPOSE: Hemophilia A (HA) is the most common X-linked inherited bleeding disorder. In some patients with HA, particularly those with severe HA, replacement therapy results in the production of high-responding clotting factor VIII inhibitors. The economic burden of this complication is the highest reported for a chronic disease. Our aim was to investigate the direct medical expenditure burden of high-responding inhibitors in patients with HA. MATERIALS AND METHODS: A retrospective study was conducted using the National Health Insurance Research Database, utilizing data covering the period of 2004-2007. RESULTS: In total, 638 males with HA, including 37 patients with high-responding inhibitors were evaluated. Over 99% of the annual median medical expenditure was attributable to the cost of clotting factor concentrates (CFCs) in patients with high-responding inhibitors. The annual median expenditure related to CFCs of the total medical care and outpatient care were US$170611 and US$141982, respectively, and were 4.6- and 4.3-fold higher in these patients during the study period, respectively. In patients with high-responding inhibitors, the median hospitalization expenditure and daily hospitalization cost with or without surgical procedures were 3.0- and 2.4-fold higher, respectively, and 4.3 and 5.6-fold higher, respectively. CONCLUSION: Our data reveal higher medical expenditures burden for patients with HA and high-responding inhibitors in Taiwan. Future research is encouraged to evaluate the impact of this burden on patient quality of life.
*Cost of Illness ; *Drug Resistance ; Factor VIII/immunology/therapeutic use ; Hemophilia A/*complications/drug therapy/economics ; Hospitalization/economics ; Humans ; Male ; *Quality of Life ; Retrospective Studies ; Taiwan