Platelet boo boos :(

Question 1

What is the type of disorder, defect, and laboratory results we would see in Von Willebrands (Type 1)

Answer 1

Disorder: Adhesion

Defect: vWF deficiency (all multimers)

Tests:
PFA = INC
PT = normal
APTT = INC (d/t DEC VIII_
PLT Aggregation = abnormal Ristocetin (will correct with addition of NP)

Question 2

What is the type of disorder, defect, and laboratory results we would see in Bernard-Soulier

Answer 2

Disorder: Adhesion

Defect: Lack of Gp1b

Tests:
PFA = INC
PLT = DEC
Large PLT
PLT Aggregation = abnormal Ristocetin

Question 3

What is the type of disorder, defect, and laboratory results we would see in Glanzmann’s

Answer 3

Disorder: Aggregation

Defect: Lack GPIIb and GPIIIa

Tests:
PFA = INC
PT = normal
APTT = normal
PLT count = normal
PLT aggregation = normal ONLY w/Ristocetin

Question 4

What is the type of disorder, defect, and laboratory results we would see in Delta Storage Pool

Answer 4

Disorder: Release

Defect: Lack of dense granules

Tests:
Hypo or agranular plts

Question 5

What is the type of disorder, defect, and laboratory results we would see in Gray Platelet

Answer 5

Disorder: Release

Defect: Marked DEC in platelet alpha granules

Tests:
Hypo or agranular plts

Question 6

What is the type of disorder, defect, and laboratory results we would see in ITP

Answer 6

Disorder: Thrombocytopenia

Defect: Spontaneous – adults/AIDS; kids/immunization

Tests:
PFA = INC
PLT count = LOW <20,000
Large plt (variable)
BM = megakaryocyte hyperplasia
Deficient clot retraction

Question 7

What is the type of disorder, defect, and laboratory results we would see in TTP

Answer 7

Disorder: Thrombocytopenia

Defect: Autoantibody against ADAMTS13, vWF cannot break apart

Tests:
- Hemolytic anemia with schistocytes
- Thrombocytopenia/hemorrhage
- Fluctuating neurological dysfunction
- Fever
- Progressive renal disease

Question 8

What is the type of disorder, defect, and laboratory results we would see in HUS

Answer 8

Disorder: Thrombocytopenia

Defect: Resembles TTP with NO neurologic symptoms. Plts are sequestered in kidney

Tests:
- MAHA
- thrombocytopenia
- acute renal failure (RBCs, protein, and casts in urine)

Question 9

What is the type of disorder, defect, and laboratory results we would see in HELLP

Answer 9

Disorder: Thrombocytopenia

Defect: Release of TXA2. Microvascular endothelial damage. Vascular lesions. Intravascular plt activation

Tests:
- Hemolysis: hgb/hct, LDH, bili, Hapt, schisto
- PLT count = LOW <200,000
- Hepatic dysfunction (AST >42, ALT >24, LDH >164)

Question 10

What is the type of disorder, defect, and laboratory results we would see in drug induced thrombocytopenia

Answer 10

Disorder: thrombocytopenia

Defect: Antibody complex attaches to platelet

Tests:
- Plt count LOW
- Test for the presence of heparin-induced ab in the patient plasma (use a plt aggregometer; if ab is present, plts will aggregate)