05 Platelet Disorders

Glanzman's

- superficial bleeding
- nl aggregation with ristocetin test
Bernard

- large platelets on smear
- abnormal ristocetin assay

Wiskott- Aldrich

Acquired
- ITP
- TTP
- HUS
- DIC
- Uremia
- thrombocytopenia
- vWF
- Hayde's
ITP

- don't live as long as they should = low number
- macrophages clear antibody bound platelets


- platelet thrombi: platelet low, coagulation factors not consumed
TTP

- much more dangerous
- low platelets, thrombosis
- thrombus form in small vessels, consumes platelets, thrombocytopenia



- TTP: build up of platelets and RBC, obstruction


- small vessel occlusion in CNS

- thrombi primarily made of platelets, do not consume clotting factor

- platelet thrombi: platelet low, coagulation factors not consumed

HUS

- distinguish from TTP: no fever or CNS symptoms (TTP only with kidney)

- platelet thrombi: platelet low, coagulation factors not consumed
DIC

- both platelets and fibrin, increased PT, PTT

- TF activate coagulation cascade
- sepsis: high levels of cytokines and endotoxins


- every blood test abnormal


- platelet thrombi: platelet low, coagulation factors not consumed
Uremia

- caused by toxins, not platelets
Thrombocytopenia


Von Willebrand



- heavy periods common early presentation


- ristocetin test normal in glanzman
- ristocetin test also abnormal in Bernard

Hayde's

- aortic stenosis: increased risks of angiodysplasia
- overactivity of ADAMST13, breaking down multimers, causing VWF to be less efficient
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