Haemolytic anaemia

Question 1

name 5 inherited haemolytic anaemia

Answer 1

• Hereditary Spherocytosis
• Hereditary Elliptocytosis
• Thalassaemia
• G6PD deficiency
• Sickle cell anaemia

Question 2

3 features of haemolytic anaemia

Answer 2

• anaemia
• splenomegaly (due to spleen filling with destroyed blood cells)
• jaundice

Question 3

blood film result in haemolytic anaemia

Answer 3

schistocytes

Question 4

direct Coombs test in autoimmune haemolytic anaemia?

Answer 4

positive

Question 5

Hereditary Spherocytosis inheritance?

Answer 5

autosomal dominant

Question 6

shape of red blood cell in hereditary spherocytosis (from what to what)

Answer 6

biconcave disc to sphere shaped

Question 7

what can precipitate aplastic crisis?

Answer 7

Parovirus infection

Question 8

presentation of Hereditary Spherocytosis? (4)

Answer 8

• jaundice
• splenomegaly
• gall stones
• aplastic crisis

Question 9

MCHC & reticulocytes in Hereditary Spherocytosis?

Answer 9

high MCHC
high reticulocytes (rapid turnover of cells)

Question 10

management of Hereditary Spherocytosis

Answer 10

• folate supplementation

- splenectomy

Question 11

inheritance of G6PD Deficiency?

Answer 11

x linked recessive

Question 12

triggers for G6PD Deficiency crises? (3)

Answer 12

• infections
• fava beans
• medications

Question 13

what is seen on blood film with G6PD Deficiency?

Answer 13

Heinz bodies

Question 14

presentation of G6PD Deficiency?

Answer 14

• neonatal jaundice
• gall stones
• anaemia
• splenomegaly

Question 15

medications that trigger G6PD Deficiency related haemolysis?

Answer 15

• primaquine
• ciprofloxacin
• sulfonyureas
• sulfasalazine

Question 16

2 types of autoimmune haemolytic anaemia? and why

Answer 16

warm & cold

depending on the temperature at which the autoantibodies that attack the red blood cells function at

Question 17

causes of Warm Type Autoimmune Haemolytic Anaemia

Answer 17

idiopathic

Question 18

what is cold autoimmune haemolytic anaemia also called?

Answer 18

cold agglutinin disease

Question 19

what is cold AIHA often secondary to?

Answer 19

• Lymphoma
• Leukaemia
• lupus
• infections: CMV, EBV, HIV, mycoplasma

Question 20

management of AIHA

Answer 20

• blood transfusions
• steroids
• rituximab
• splenectomy

Question 21

Rituximab mechanism

Answer 21

Monoclonal antibody against B cells

Question 22

Inheritance of sick cell anaemia?

Answer 22

Autosomal recessive

Question 23

when is fetal haemoglobin (HbF) usually replaced by Haemoglobin A

Answer 23

6 weeks of life

Question 24

abnormal gene in sickle cell anaemia?

Answer 24

gene for beta globin, on chromosome 11

Question 25

what is the relation between sickle cell anaemia & malaria

Answer 25

having one copy of the gene/sickle cell trait, makes you more likely to survive malaria = selective advantage in malaria prevalent areas (e.g. Africa, India, Middle east, carribean)

Question 26

triggers of vaso-occlusive sickle crisis? (4)

Answer 26

- cold - dehydration - infection - hypoxia

Question 27

general management of Sickle cell anaemia

Answer 27

- maintain hydration & warmth - phenoxymethypenicillin - Hydroxycarbamide - blood transfusion - bone marrow transplant

Question 28

how to prevent infections in sickle cell patients?

Answer 28

Penicillin V/Phenoxymethylpenicillin OD

Question 29

Management of sickle cell crises?

Answer 29

- admit - treat infection - keep warm & maintain hydration - analgesia - priapism

Question 30

role of priapism in sick cell crises?

Answer 30

in a crises, the sickle blood cells clump together and block/affect the blood flow in penis... to manage this; 'priapism' - blood aspiration

Question 31

what is a vaso-occlusive crisis?

Answer 31

sickle shaped blood cells clog up capillaries causing reduced blood perfusion distal to the blockage --> ischaemia

Question 32

what happens in a splenic sequestration crisis?

Answer 32

red blood cells clump together and block blood flow within spleen. The pooling of blood in the spleen can lead to a severe anaemia and circulatory collapse.

Question 33

what is aplastic crisis & what triggers it?

Answer 33

- temporary loss of creation of new RBC | - triggered by Parovirus B19 infection

Question 34

what does Parovirus B19 infection trigger

Answer 34

aplastic crisis

Question 35

diagnostic critera for acute chest syndrome (2)

Answer 35

- fever/ resp symptoms | - new infiltrates seen on x-ray

Question 36

management of acute chest syndrome (4)

Answer 36

1) infection management: abx/antivirals 2) blood transfusion 3) NIV 4) incentive spirometry