Coagulation Disorders
Must know Coagulation Cascade!!!
Factor V Leiden
MC hypercoagulability inherited disorder
Protein C acts as a stop sign to break down Factor V
MUTATED Factor V with no red light (therefore will continue to contribute to clotting)
**DVT and PE’s in young patients**
Protein C Deficiency
Autosomal Dominant
Protein C acts as a stop sign to break down Factor V
NORMAL Factor V with no red light (therefore will continue to contribute to clotting)
**DVT and PE’s in young patients**
Hemophilia A
Factor 8 deficiency
Blood in the joints = **HEMARTHROSIS**
**PROLONGED PTT** (Other studies are normal)
Tx: Factor 8 , DDAVP
Hemophilia B
**CHRISTMAS DISEASE**
Factor 9 deficiency
Blood in the joints = **HEMARTHROSIS**
**PROLONGED PTT** (Other studies are normal)
Tx: Factor 9 (DDAVP not useful)
Von Willebrand Disease
*MC hereditary bleeding disorder*
vWF deficiency affecting platelet plug and Factor 8
Dx: **DECREASED RISTOCETIN ACTIVITY**, decreased vWF, decreased Factor 8
Tx: Depends on severity
PANCE Prep Pearls has an incredible chart that is worth memorizing for all the differences in PT, PTT, Bleeding Time, and Platelet Count for each bleeding disorder
Idiopathic Thrombocytopenia Purpura (ITP)
Low platelet count
(Look for the kid with the excessive gum bleeding or nose bleeds after a URI)
MC in kids are viral illness
Tx: Observation vs Steroids (in adults) vs IVIG vs Splenectomy
Hemolytic Uremic Syndrome (HUS)
Diarrhea —-> R. A. T.
Gastroenteritis in kids leading to Renal Failure, Anemia, Thrombocytopenia
Tx: Fluids (ABX not indicated)
Thrombotic Thrombocytopenia Purpura (TTP)
The following is a MNEMONIC strictly for memorization purposes and has no malice intent
Tx: Plasmaphoresis due to autoimmune reaction of disease **(Decreased ADAMTS13)**
PANCE Prep Pearls has an incredible chart that is worth memorizing for all the differences in ITP, TTP, HUS, and DIC.
Disseminated Intravascular Coagulation (DIC)
Over exaggeration of bodies clotting mechanism causing microthrombi then once all clotting resources are used up excessive bleeding occurs
(Look for the post C-section patient with extensive bleeding)
TREAT UNDERLYING CAUSE
For additional questions and explanations click below!
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