The Haemolytic Anemias

Question 1

Most hemoglobin degradation occurs in the

Answer 1

Macrophages of the spleen

Question 2

Indirect indicators of erythrocyte destruction

Answer 2

Blood bilirubin
Urobilinogen concentration in the urine

Question 3

Bilirubin is conjugated in the liver with

Answer 3

Glucoronic acid
Enzyme-Glucoronyl transferase

Question 4

Haemolysis is associated with (bilirubin)

Answer 4

High concentrations of UNCONJUGATED BILIRUBIN

Question 5

Evidence of increased haemolysis: Biochemical consequences

Answer 5

Hyberbilirubinemia(unconjugated)
Reduced serum haptoglobin

Question 6

Evidence of increased haemolysis: intravascular haemolysis

Answer 6

Haemoglobinemia
Reduced serum haptoglobulin
Reduced serum hemopexin
Haemoglobinuria
Haemosiderinuria
Methalbuminemia (Schumm’s test)

Question 7

Evidence of damaged red cells

Answer 7

Micro-spherocyte
Red cell fragments
Sickle cells

Question 8

Evidence of increased red cell production

Answer 8

Polychromasia
Nucleated red cells

Question 9

Clinical presentations of haemolysis

Answer 9

Jaundice
Anemia

Question 10

Clinical features of haemolysis states

Answer 10

1. Jaundice
2. Pallor
3. Pigment stones
4. Splenomegaly
5. Expansion of marrow cavities in congenital HA

Question 11

Laboratory evidence of increased erythropoietic activity

Answer 11

1. Reticulocytosis (polychromasia)
2. Erythroblastaemia (nucleated rbcs)
3. Macrocytosis (High MCV)
4. Erythroid hyperplasia (Bone marrow)
5. Reduced myeloid/erythroid ratio (Bone marrow)
6. Changes in the skull and tubular bones

Question 12

Evidence of red cell damage

Answer 12

Spherocytes
Fragment cells

Question 13

Reticulocyte count is increased/decreased in hemolysis

Answer 13

Increased

Question 14

Hemosiderinuria and Hemoglobinuria are present in

Answer 14

INTRAVASCULAR hemolysis

Question 15

Complications of Hemolysis

Answer 15

1. Aplastic crisis
2. Leg ulcers; chronic haemolytic states

Question 16

Diagnosis of hemolysis

Answer 16

1. Demonstration of a hemolytic state
2. Demonstration of its aetiology

Question 17

Haemolytic states

Answer 17

Polychromasia +/- NRBC
Reticulocytosis
Erythroid hyperplasia

Question 18

Treatment of haemolysis

Answer 18

Treat the underlying cause
Give folic acid
Red cell concentrates

Question 19

Structure of the red cell membrane

Answer 19

Trilaminar
Outermost (glycolipids, glycoproteins)
Central (cholesterol, phospholipids)
Inner (cytoskeleton)

Question 20

Most common congenital hemolytic anemia

Answer 20

Hereditary Spherocytosis

Question 21

Inheritance is

Answer 21

Autosomal recessive/Autosomal dominant

Question 22

Clinical features of Hereditary spherocytosis

Answer 22

Asymptomatic
Episodic jaundice
Variable splenomegaly
Gall stones

Question 23

Laboratory features of hereditary spherocytosis

Answer 23

1. Negative antiglobulin test
2. Variable degree of spherocytosis
3. Floe cytometric analysis for EMA binding to detect spherocytes
4. Membrane protein defect test -
   SDS-PAGE, RIA or EIA

Question 24

Diagnosis of hereditary spherocytosis

Answer 24

Can be missed till adulthood

Question 25

Molecular defect of Hereditary Spherocytosis

Answer 25

1. RBC membrane loss is due to defective vertical protein linkage between membrane skeleton and lipid bilayer 2. Caused by molecular defects in genes encoding for proteins (spectrin, ankyrin, band 3 and protein 4.2)

Question 26

Molecular defect of hereditary Elliptocytosis

Answer 26

Most common defect is in soectrin

Question 27

Clinical features of hereditary elliptocytosis

Answer 27

Asymptomatic Anemia No splenomegaly

Question 28

Differentials of H.E

Answer 28

Iron deficiency Artifacts Myelodysplasia

Question 29

Inheritance of G6PD deficiency

Answer 29

X linked, recessive (commoner in males)

Question 30

Feature of G6PD Deficiency episodic haemolysis

Answer 30

Acute haemolysis induced by increased oxidant stress

Question 31

G6PD Deficiency: Triggers of acute haemolysis

Answer 31

1. Antimalarials, sulphonamides, nitrofurans 2. Favism 3. Infections

Question 32

Clinical features of G6PD Deficiency

Answer 32

Acute haemolysis Neonatal jaundice (most common cause)

Question 33

Laboratory features of G6PD deficiency

Answer 33

Anisocytosis Bite cells Heinz bodies

Question 34

Treatment of G6PD deficiency

Answer 34

1. Stop precipitating drug or treat the infection 2. Acute transfusions, if possible

Question 35

Types of immune haemolytic anaemia

Answer 35

Autoimmune Alloimmune

Question 36

Types of autoimmune haemolytic anaemia

Answer 36

Warm IHA Cold IHA Drug induced IHA

Question 37

Causes of Warm AI Haemolysis

Answer 37

50% Idiopathic SECONDARY 1. Lymphoid neoplasm e.g CLL, Lymphoma, Myeloma 2. Solid tumors eg Lung, Kidney 3. Connective tissue ds e.g Lupus, RA 4. Drugs e.g Alpha methyl DOPA, Penicillin, Quinine, Chloroquine 5. Misc e.g UC, HIV

Question 38

Types of CAHA

Answer 38

Cold agglutinin syndrome (CAD) Paroxysmal cold hemoglobinuria

Question 39

Causes of secondary CAD

Answer 39

Mycoplasma Infectious mono LPD

Question 40

Causes of secondary Paroxysmal Cold Hemoglobinuria

Answer 40

Measles Mumps Syphilis

Question 41

Who is affected by Warm AI Haemolysis

Answer 41

More females than males All age groups

Question 42

P smear of Warm AI Hemolysis

Answer 42

Micro-spherocytosis Nucleated RBCs

Question 43

Confirmation of Warm AI Haemolysis

Answer 43

Coombs test Antiglobulin test

Question 44

Treatment of Warm AI haemolysis

Answer 44

Correct underlying cause 1. Prednisolone 2. Transfusion; for severe occasions 3. Splenectomy (if steroids don’t work) 4. Immunosuppressives: Azathioprine, Cyclophosphamide

Question 45

Clinical features of CAD

Answer 45

Acrocyanosis

Question 46

IgM with specificity to I or IAg is seen in what disease

Answer 46

Cold AI Haemolysis

Question 47

Examples of Mechanical trauma to red cells in Non immune HA

Answer 47

1. Abnormalities to heart and large vessels 2. Microangiopathic HA - HUS, TT, DIH 3. March haemoglobinemia