Bleeding disorders

Question 1

abnormalities of what give bleeding disorders?

Answer 1

Any quantitative or qualitative abnormality, Inhibition of function: Platelets, vWF, Coagulation factors.

Question 2

what to ask in a hx of bleeding?

Answer 2

PATTERN, CONGENITAL, SEVERITY? Bruising, Epistaxis, Post-surgical bleeding, Menorrhagia, Post-partum haemorrhage, Post-trauma

Question 3

Patterns of bleeding

Answer 3

Platelet type: Mucosal (purpura), Epistaxis, Purpura, Menorrhagia, GI
Coagulation Factor Articular: Muscle Haematoma, CNS - intracranial haemorrhage in haemophilia

Question 4

Haemophilia A and B

Answer 4

X linked, severity of bleeding depends on residual coagulation factor activity. A is a F8 deficiency, B is a F9 deficiency. A is more common.

Question 5

Px HA and HB

Answer 5

Haemarthrosis - knees, albows and ankles
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding

Question 6

clinical complications of haemophilia

Answer 6

Synovitis, Chronic Haemophilic Arthropathy, Neurovascular compression (compartment syndromes), Other sequelae of bleeding (Stroke)

Question 7

Ix haemophilia

Answer 7

↑APTT, normal PT, ↓F8/9 assay

Question 8

Tx haemophilia

Answer 8

Coagulation factor replacement, Avoid NSAIDs and IM injections, Minor bleeds: desmopressin + tranexamic acid, Major bleeds: rhF8.
Splints, Physiotherapy, Analgesia, Synovectomy, Joint replacement, gene therapy and testing (amniocentesis, chorionic villus sampling) DDAVP (causes the release on vwf into circulation)

Question 9

complicaitons of Haemophilia tx…

Answer 9

Viral infection: HIV, HBV, HCV, Others/ vCJD?
Inhibitors: Anti FVIII Ab, Rare in FIX,
DDAVP: MI, Hyponatraemia(babies) - people woth cardiac issues and young children shouldn’t have DDAVP because it increases water retention

Question 10

VON WILLEBRAND DISEASE

Answer 10

Commonest inherited clotting disorder (mostly AD), (mucosal) Platelet type bleeding.

Question 11

VON WILLEBRAND DISEASE quantitative is type 1 and is…

Answer 11

depends on the amount of vwf

Question 12

VON WILLEBRAND DISEASE qualitative is type 2 and is…

Answer 12

depends where the site of the mutation is

Question 13

tx VWD

Answer 13

desmopressin + tranexamic acid, vWF concentrate or DDAVP

Question 14

what causes a decreased productiopn of platelets in thrombocytopenia?

Answer 14

marrow failure, aplasia, infiltration, drugs, egaloblastic anaemia.

Question 15

what causes a increased consumption of platelets in thrombocytopenia?

Answer 15

immune ITP, non immune DIC, hypersplenism.

Question 16

Sy/Sx of thrombocytopenia

Answer 16

bleeding from structural lesions - varices, petechia, eccymosis, mucosal bleeding, cns bleeding is rare.

Question 17

ITP

Answer 17

Children: commonly post-URTI, self-limiting
Adults: F>M, long-term
Ix: Anti-platelet Abs present
Rx: conservative, steroids, IVIg, splenectomy

Question 18

what is given straight away to newborns?

Answer 18

Vitamin K - this prevents haemorrhagic disease of the newborn.

Question 19

DDx for haemophilia A

Answer 19

VonWillebrands disease, factor 5 leiden, antiphospholipid syndrome.

Question 20

how is Haemophilia A inherited genetically?

Answer 20

X linked, severity of bleeding depends on residual coagulation factor activity. A is a F8 deficiency, B is a F9 deficiency. A is more common.

Question 21

mx of Haemophilia?

Answer 21

prophylaxis with factor 8, training for when bleeding occurs at home, avoid contact sports, accident proofing.