A Case: 50 yo M past medical history cancer on chemotherapy presents with fever and altered mental status.
His labs are concerning for a Cr 7, Hgb 5 (from 12), platelet’s 30
Diagnosis?Thrombotic Thrombocytopenic Purpura (TTP)
Classical presentation of this disease?
FAT RN – Fever, microangiopathic hemolytic Anemia (schistocytes), Thrombocytopenia, Renal failure, Neurologic abnormalities. Very rare to have all 5.
Treatment?
Steroids, plasma exchange transfusion, FFP
What treatment is contra-indicated and why?
Platelet transfusion as the platelet’s will get broken apart leading to more clots
For double-extra points/for nerds, what is the pathophysiology?
Though a genetic cause does exists, TTP can develop from acquired inhibition of ADAMTS13. This protein typically breaks down Von Willebrand’s Factor. Without ADAMTS13, increasingly large vWF leads to increased platelet adherence to vessel wall and shearing of RBC’s.
By Dr. Andrew Grock and Dr. Sally Bogoch
Rivers, 6th edition.
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