Hemolytic Anaemia

Question 1

Haemosiderinuria is a very valuable sign ……..

Answer 1

Of chronic intravascular hemolysis

Question 2

Frontal bossing or hair-on-end appearance is a classical sign of ………..

Answer 2

Bone marrow hypercellularity

Question 3

What is Aplastic crisis?

Answer 3

Aplastic crisis in commonly seen in px with chronic inherited hemolytic anaemia
Associated with infection of BFU E with parvovirus B19

Rarely in foliate deficiency in megaloblastic anaemia

There’s reticulocytopaenia

Question 4

Actin is composed of

Answer 4

Tropomyosin
Tropomodulin
Adducin
Dematin

Question 5

The forms of membranopathies include

Answer 5

Hereditary spherocytosis
Hereditary elliptocytosis
Hereditary pyropoikilocytosis
Hereditary stomatocytosis
Hereditary acanthocytosis
South East Asian ovalocytosis

Question 6

What is the triad of HS

Answer 6

Spherocytosis
Osmotic fragility
Dominant inheritance

Question 7

What are the morphological features of HS

Answer 7

Spherical shape
No central area of pallor
Smaller diameter

Question 8

What is the commonest defect in HS

Answer 8

Spectrin deficiency

The greater the deficiency the greater the spherocytosis

The greater the degree of osmotic fragility
The severe the hemolysis

Question 9

The usual feature of HS is

Answer 9

Mild to moderate hemolytic anaemia

Question 10

The classical clinical features of HS is

Answer 10

Mild to moderate hemolytic anaemia

Splenomegaly
Bile stones
Aplastic crises

Question 11

Jaundice is typically marked in HS when it is associated with …

Answer 11

Gilbert’s disease

Question 12

………… Hereditary hemolytic anaemia is described as more yellow than sick

Answer 12

Hereditary spherocytosis

Question 13

In order of frequency the most common clinical features of HS are

Answer 13

Jaundice
Moderate splenomegaly
Anaemia

Question 15

During the pathophysiology of sickle cell anaemia after the formation of heterogeneous nucleation ……….. are formed in which order

Answer 15

Tactoid fibrils then fibre

Question 17

The GPI anchor proteins are …… , …… and thy are …..

Answer 17

CD55
CD59

DAF and MIRL

Decay accelerating factor and membrane inhibitor of reactive lysis

Question 19

What is red cell fragmentation syndrome?

Answer 19

It is the physical damage to red cells in circulation

Characterized by schistocytes
Can take up different shapes triangular, helmet, comma

Question 20

Describe the morphology of schistocytes……

Answer 20

Microcytic with no area of pallor and still has the same deformability like normal rbcs

Question 21

The normal range of schistocytes are …..

Answer 21

0.2-0.5%

Question 22

What is the triad of hemolytic uraemic syndrome

Answer 22

Intravascular hemolysis
Renal