Haematology 1: Haemolytic Anaemias

Question 1

What is the difference between intravascular and extravascular haemolysis ?

Answer 1

Intravascular- occurs inside the circulation

Extravascular- Occurs in the reticuloendothelial system

Question 2

Which one of these is an example of a disease in which you get extravascular haemolysis ?

A) Malaria
B) Hereditary spherocytosis
C) G6PD
D) Microangiopathic haemolytic anaemia

Answer 2

B- hereditary spherocytosis

Occurs in the spleen.
The others are all intravascular

Question 3

Name one drug that causes intravascular haemolysis ?

Answer 3

Dapsone

Question 4

What is the inheritance pattern of Hereditary spherocytosis ?

Answer 4

Autosomal dominant

Question 5

What happens in haemolytic anaemia patients if they become infected with Parvovirus b19 ?

Answer 5

Transient aplastic crisis

Massive reduction in Hb and circulating RBCs. Low reticulocytes shows RBC production has stopped

Question 6

What is the Name of the disease caused by Parvovirus b19 infection in children ?

Answer 6

Fifth’s disease

Question 7

“Slapped cheeks” rash is characteristic of which infection in children ?

Answer 7

Parvovirus B19 (fifth’s disease)

Question 8

What disease can immunocompromised patients get if they become infected with Parvovirus B19 ?

Answer 8

Pure red cell aplasia

Question 9

Which stain can detect hepatic siderosis (iron overload)?

Answer 9

Perl’s stain (Prussian blue)

Question 10

What are haptoglobins ?

Answer 10

A Protein in the blood that binds to and removes free haemoglobin

Question 11

What causes Paroxysmal nocturnal haemoglobinuria ?

Answer 11

Acquired genetic defect in GPI anchor

Question 12

What does a low level or absent haptoglobin suggest ?

Answer 12

Intravascular haemolysis

All the haptoglobins have been used up and there is lots of free haemoglobin

Question 13

Which of these proteins is not part of the red cell membrane ?

A)Spectrin
B)Ankyrin-1
C)Band 3
D) GPI
E) Myosin

Answer 13

E) Myosin

Question 14

What is the diagnostic test for Hereditary spherocytosis ?

Answer 14

Osmotic Fragility test

Question 15

Which autosomal recessive disease similar to hereditary eliptocytosis has abnormal sensitivity to heat?

Answer 15

Hereditary Pyropoikilocytosis

More severe

Question 16

How is G6PD inherited ?

Answer 16

X-linked

Question 17

List 6 triggers of acute haemolysis in patients with G6PD

Answer 17

Antimalarials 
Dapsone
Favs beans 
Napthalene moth balls 
Infections
Antibiotics (Sulphonamides)

Normally G6PD patients are asymptomatic but these agents are oxidative and cause acute haemolysis

Question 18

Name 2 characteristic cells seen in G6PD ?

Answer 18

Bite cells

Hemighosts

Question 19

Oxidative haemolysis causes characteristic Blue inclusions of denatured haemoglobin in RBCs. These are also known as ………………………

Answer 19

Heinz bodies

Question 20

Name one type of cell that is characteristically seen in PKD (pyruvate kinase deficiency) ?

Answer 20

Burr cells (echinocytes)

They are spikey cells that look like sea urchins

Question 21

List 4 causes of basophilic stippling ?

Answer 21

Pyrimidine 5 nucleotidase deficiency
Lead poisoning
Megaloblastic anaemia (alcoholics)
Thalassaemia

Question 22

Which test is used to diagnose Paroxysmal nocturnal haemoglobinuria (PNH) ?

Answer 22

HAM’s test

Question 23

Which test is used to diagnose Malaria ?

Answer 23

Thick and thin blood films

Question 24

Which test is used to diagnose Autoimmune haemolytic anaemia ?

Answer 24

DAT test

Question 25

Which type of immunoglobulin causes warm autoimmune haemolytic anaemia ?

Answer 25

IgG

Question 26

Which type of immunoglobulin causes cold auto immune haemolytic anaemia ?

Answer 26

IgM

Question 27

Child recovering from measles suddenly presents with haemoglobinuria after going into an ice bath. Complement levels are low. Donath-Landsteiner test is positive. Most likely diagnosis ?

Answer 27

PCH (paroxysmal cold haemoglobinuria) Preceding viral infection Cold exposure IgG antibodies bind to P-antigen on red cells Causes complement mediated haemolysis on warming up again

Question 28

Which test can be used to diagnose Paroxysmal nocturnal haemoglobinuria ?

Answer 28

Ham’s test | Or immunophenotyping

Question 29

Which drug can be used to treat PNH?

Answer 29

Eculizumab

Question 30

Which antibodies cause TTP ?

Answer 30

Antibodies against ADAMTS13 (ADAMTS13 is an enzyme which normally cuts vWF into smaller strands of protein. In TTP, very long strands of vWF cause shearing of RBCs in vessels leading to haemolysis and schistocytes)

Question 31

What is the Pentad for TTP ?

Answer 31

``` MAHA Thrombocytopenia Renal impairment fever Neurological symptoms ```

Question 32

Which infection is commonly responsible for causing HUS ?

Answer 32

E.Coli

Question 33

Which conditions are associated with cold AIHA ?

Answer 33

Think cold LID Lymphoproliferative - CLL, Lympomas Infections - Mycoplasma, EBV Do not know - Idiopathic

Question 34

List 2 conditions associated with Spherocytes ?

Answer 34

Hereditary spherocytosis | Warm AIHA

Question 35

List 2 conditions and 1 drug that are associated with warm AIHA ?

Answer 35

CLL SLE Penicillin

Question 36

Name a congenital cause of aplastic anaemia ?

Answer 36

Fanconi anaemia

Question 37

List 4 drugs can cause aplastic anaemia ?

Answer 37

Carbamazepine Chloramphenicol Cytotoxic drugs Phenytoin

Question 38

Name 1 drug used in the treatment of Sickle cell anaemia ?

Answer 38

Hydroxycarbamide

Question 39

Which drug acts to replenish vitamin b12 stores in pernicious anaemia ?

Answer 39

IV Hydroxocobalamine

Question 40

List 2 drugs that can cause Folate deficiency ?

Answer 40

Methotrexate | Phenytoin

Question 41

List causes of MAHA ?

Answer 41

``` TTP HUS DIC SLE Malignant hypertension Pre-eclampsia ```