Acquired hemophilia

Acquired hemophilia
Updated: 07/25/2023
© Jun Wang, MD, PhD

General features
  • Rare but potentially life-threatening bleeding disorder
  • Associated with autoimmune disorders, solid tumors, hematologic malignancies, dermatologic disorders, inflammatory bowel disease, respiratory diseases, diabetes, acute hepatitis (B and C), severe drug reactions, post-childbirth, or previous usage of coagulation factors
Clinical presentations
  • Bleeding
  • Hemarthrosis uncommon
Key pathogenesis
  • Autoantibodies (inhibitors) directed against plasma coagulation factors
  • Most frequently factor VIII (FVIII)
Key laboratory findings
  • Prolonged aPTT NOT reversed on a mixed study
  • Reduced FVIII levels and evidence of an FVIII inhibitor
  • Normal PT, platelet count
Treatment
  • Treat underlying disorder
  • Desmopressin in very low titer inhibitors
  • High-dose Factor VIII is low titer inhibitors
  • Recombinant Factor VIIa for severe bleeding


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Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4.  Benign blood cell disorders Anemia Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas...
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