Thrombotic microangiopathy assoc w/hemolytic anemia from ADAMTS13 deficiency→ prevents cleavage of von Willebrand factor→ promotes thrombosis→ organ damage & thrombocytopenia
Consumption leads to thrombocytopenia
Risk Factors
Hereditary or acquired (more common)
Adults > children for acquired; children > adults hereditary
Can be triggered by meds→ quinidine, clopidogrel, other
Also triggered by: Autoimmune Dz→ e.g. SLE; Infections→HIV, viral, bacterial, fungal, spirochete, other; Pregnancy, trauma, surgery, cancer; Stem cell transplant; Also idiopathic
Symptoms
Acute or subacute
AKI less common than w/HUS
Mucosal bleeding
Neuro issues in 50% including confusion, seizures, focal deficits; other sx based on where thrombi occur including GI, sepsis
Presentation overlaps w/many other conditions
Physical Exam Findings
Often ill appearing
Typically afebrile
Petechiae and mucosal hemorrhages
Target organ dysfxn→ focal neuro deficits, confusion from general toxicity, other
Tests
CBC w/anemia and thrombocytopenia, sometimes AKI, elevated LDH, T Bili; low Haptoglobin
Schistocytes on peripheral smear
ADAMTS13 level low
Typically INR/PT, PTT, Fibrinogen and D-Dimer normal (unless another process is contributing)