Haem- bleeding disorders

Question 1

what is haemophilia

Answer 1

An X-linked recessive, hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion

Question 2

haemophilia aetiology

Answer 2

haemophilia A- factor VIII deficiency
haemophilia B- factor IX deficiency

Question 3

what is more common haemophilia A/haemophilia B?

Answer 3

haemophilia A- 5x more common

Question 4

haemophilia affects which vessels

Answer 4

Bleeding from medium to large blood vessels, most commonly into joints

Question 5

what are haemarthroses

Answer 5

bleeding into a joint space. It can cause pain, swelling, and limited joint movement

Question 6

blood results in haemophilia

Answer 6

Isolated prolonged APTT
Coagulation factor assays (factor VIII for A, factor IX for B)

Question 7

Desmopressin moa

Answer 7

stimulates the release ofvon Willebrand Factor

Question 8

Von Willebrand factor

Answer 8

a protein in the blood that helps blood clot

Question 9

what is von willebrand’s disease

Answer 9

Inherited bleeding disorder characterized by a reduced quantity or function of von Willebrand factor

Question 10

VWF gene is located on chromosome __

Answer 10

12

Question 11

what is immune thrombocytopenic purpura

Answer 11

Clinical syndrome in which a decreased number of circulating platelets manifests as a bleeding tendency, causing petechiae, ecchymoses, purpura and bleeding from venepuncture sites, nose etc., in an otherwise healthy patient

Question 12

Causes of secondary ITP

Answer 12

other autoimmune disorders- antiphospholipids syndrome and SLE
infections- HIV etc
H.pylori infection
medication
Lymphoproliferative disorders

Question 13

which type of hypersensitivity reaction is ITP

Answer 13

type II hypersensitivity reaction

Question 14

ITP is caused by antibodies that target and destroy what?

Answer 14

platelets

Question 15

which age group of patients does ITP usually affect

Answer 15

children under 10 years old

Question 16

first line treatment for ITP when treatment required

Answer 16

oral prednisolone

Question 17

what is thrombotic thrombocytopenic purpura

Answer 17

Rare form of thrombotic microangiopathy characterised by microangiopathic haemolysis, thrombocytopenia, and neurological abnomalities

Question 18

TTP caused by a deficiency of what

Answer 18

metalloproteinase ADAMTS13

Question 19

TTP associated with what triggers

Answer 19

medication
AIDS
malignancy

Question 20

Classic pentad of TTP

Answer 20

fever
Microangiopathic haemolytic anaemia
Thrombocytopaenic purpura
CNS involvement: headache, confusion, seizures
AKI

Question 21

what is Microangiopathic haemolytic anaemia

Answer 21

a type of anemia that occurs when red blood cells break down in small blood vessels. It’s characterized by the presence of schistocytes, which are fragments of red blood cells, in a blood sample

Question 22

definitive treatment of TTP

Answer 22

plasma exchange

Question 23

blood film findings in TTP

Answer 23

schistocytes
raised reticulocyte count

Question 24

what is a massive haemorrhage

Answer 24

bleeding patient with signs of shock
bleeding which has already prompt emergency O Rh(D) negative red cells

Question 25

most appropriate medical management for von willebrand’s disease

Answer 25

Desmopressin

Question 26

most common inherited bleeding disorder

Answer 26

von willebrand’s disease

Question 27

disseminated intravascular coagulation (DIC)

Answer 27

Complex condition that describes the inappropriate activation of the clotting cascades, resulting in thrombus formation and subsequently leading to the depletion of clotting factors and platelets

Question 28

causes of DIC

Answer 28

sepsis obstetric emergencies malignancy hypovolaemic shock