Other disorders Flashcards
What mutation causes polycythemia vera?
janus kinase 2, a cytoplasmic tyrosine kinase
DDX of aquagenic pruritis, facial plethora, and splenomegaly?
polycythemia vera
What infection is polyarteritis nodosa associated with?
hepatitis B. PAN sx = fever, ab pain, periph neuropathy, weakness, weight loss
What substance is the functional opposite of thromboxane A2, and produced by endothelial cells?
prostacyclin
What is kallikrein?
converts kininogen into bradykinin
What is hageman factor?
factor XII of coag pathway
What 2 vasculidities exhibit granulomatous inflammation of the media?
Takayasu arteritis and giant cell arteritis
What 3 vasculidities are characterized by fibrinoid necrosis of small vessels?
microscopic polyarteritis, hypersensitivity vasculitis, microscopic polyangiitis. also rheumatoid does this to any size vessel
Which vasculitis exhibits transmural necrotizing inflammation of medium to small sized arteries?
polyarteritis nodosa
What vasculitis exhibits thrombosis of medium and small arteries, particularly the tibial and radial?
thromboangiitis obliterans, aka buerger’s disease
What factor predominantly is associated with Buerger’s disease?
smoking
Antibodies are produced against what protein in diffuse scleroderma?
DNA -topoisomerase
Why does treatment with vasopressin in Vonwildebrand’s deficiency result in increased clotting?
It induces release of vWF from endothelial cells within 30-60 min of admin. only works for pts with mild def, obvi
What protein is absent or defective in Glanzmann’s thrombasthenia?
GPIIb/IIIa
What factor does von Willebrand factor protect and carry?
factor VIII
