Hemmorhagic diseases

Liver Disease Coagulopathy

FVII Priority

Liver Disease Coagulopathy

Factor V Isolation

Liver Disease Coagulopathy

Fibrinogen = <100 mg/dl (end-stage liver failure)

Vitamin K Deficiency

broad-spectrum antibiotics (killing gut flora)

Liver Disease Coagulopathy

chronic or compensated DIC.

Vitamin K Deficiency

coumarin/warfarin therapy (blocking Vit K recycling)

Vitamin K Deficiency

brodifacoum/superwarfarin (rat poison) ingestion

Vitamin K Deficiency

Hemorrhagic Disease of the Newborn:

Chronic Renal Failure (CRF) Hemorrhage

Adequate platelet numbers, but severe functional platelet defect.

Chronic Renal Failure (CRF) Hemorrhage

Nephrotic Syndrome Variant: extrinsic factors are physically lost in the urine, leading to a prolonged PT with or without a prolonged aPTT.

Chronic Renal Failure (CRF) Hemorrhage

BT - prolonged (unrelible)
Plt aggregmetry - nonproductive
PT and APTT - normal

Auto-Anti-Factor VIII Inhibitor & Acquired Hemophilia A (AHA)

An elderly patient with no prior personal or family history of bleeding presenting with sudden, severe deep tissue or gastrointestinal bleeding. Often associated with autoimmune diseases (SLE, RA) or triggered by pregnancy.

Kinetics (Nijmegen-Bethesda Assay)

quantitate inhibitor levels

Kinetics (Nijmegen-Bethesda Assay)

Treated with FEIBA (FVIII inhibitor bypassing activity)

Seen in Congenital Severe Hemophilia A patients who develop alloantibodies.

Kinetics (Nijmegen-Bethesda Assay)

true Acquired Hemophilia A

Congenital VWD

moderately prolonged aPTT if FVIII carrier capacity drops below 40 IU/dl

Acquired VWD

Hypothyroidism and WIlms tumor or SLE

Hemophilia C (Rosenthal Syndrome)

intrinsic pathway deficiency of Factor XI. Characterized by a prolonged aPTT

Factor XIII Inhibitors

Life-threatening bleeding linked to Isoniazid

Factor V Autoantibodies:

Arise spontaneously or after surgical exposure to bovine thrombin in fibrin glue

Factor X Autoantibodies

Critically rare; strongly associated with systemic amyloidosis.