Bleeding disorders

Question 1

Thrombocytopenia

Answer 1

Platelets < 30 -50 * 10^9/L

Question 2

Trhombocytopathia

Answer 2

Defective platelet function

Question 3

Bernard-Soulier Syndrome

Answer 3

Autosomal recessive

GpIb defect, not able to bind vWF

Question 4

Glanzmann thrombasthenia

Answer 4

Autosomal recessive
GpIIb/IIIa defect, no platelet aggregation
More serious than Bernard-Soulier

Question 5

Storage pool disease

Answer 5

Reduction in number or content of platelet granules

Question 6

Abnormal primary hemostasis

Answer 6

Incraesded bleeding time
Normal PT and aPTT
Petechiae, ecchymosis, spontaneous bleedings mucosal surfacs

Question 7

Abnormal secondary hemostasis

Answer 7

Normal bleeding time
Prolonged PT and/or aPTT
Large bleedings muscles and joints, re-bleeding

Question 8

Von Willebrand Disease

Answer 8

Autosomal inheritance
Men:Women
Abnormality both 1ry and 2ry hemostasis

Question 9

Type 1 VWD

Answer 9

Less vWF, function normal

Question 10

Type 2a VWD

Answer 10

HMW mulitmer deficiency > decreased platelet binding

Question 11

Type 2b VWD

Answer 11

Increased affinity of VWF for GpIIb > spontaneous clumping

Question 12

Type 2m VWD

Answer 12

Deficiency receptorbinding > decreased platelet binding

Question 13

Type 2n VWD

Answer 13

Decreased affinity vWF for VIII > defect 2ry hemostasis

Question 14

Type 3 VWD

Answer 14

Complete deficiency of vWF

Question 15

Normandy VWD

Answer 15

Less binding of VIII

Binding of vWF to VIII is required for survival of VIII

Question 16

Clinical presentation VWD

Answer 16

Skin and mucosal bleedings
Nosebleeds
Menorrhagia
Bleeding after trauma and surgery
Less severe bleeding tendency than hemophilia

Question 17

Hemophilia

Answer 17

X linked hereditary disease, men affected women carriers

Question 18

Hemophilia A

Answer 18

Shortage of VIII

Question 19

Hemophilia B

Answer 19

Shortage of IX

Question 20

Mild hemophilia

Answer 20

Bleeding after trauma and surgery

Question 21

Moderate hemophilia

Answer 21

Sometimes spontaneous bleeding

Bleeding after trauma and surgery

Question 22

Severe hemophilia

Answer 22

Spontaneous bleeding

Bleeding after trauma and surgery