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Week 4 – Handout

1. Normal to decreased Hb, Increased RBC, Decreased MCV & MCH,


moderate microcytosis, Mild target cells and elliptocytes, mild
polychromasia and basophilic stippling. HbF slightly increased, HbA1
present.

What is your provisional diagnosis?

 Also called β-Thalassaemia trait


 One of the two genes that produce β-globin chains is defective
(heterozygous)
 Asymptomatic
 Often confused with iron deficiency anaemia

Hb Normal - Decreased
Increased
RBC (Marrow compensating for ineffective
haemopoiesis)
MCH & MCV Decreased
Hypochromasia Mild
Anisocytosis Moderate Microcytes
Poikilocytosis Mild Target Cells +, mild elliptocytes

Immature Forms Polychromasia, basophilic stippling (wrights stain)


 Clinically well except when: Stressed, Infected or Pregnant

2. 65 y old female, transfusion dependant. Hb is 85 g/L. MCV and MCH is


low. RBC count slightly low. Dimorphic blood film observed.

Comment on the morphology. What are the characteristic features you


find in this film? What is your provisional diagnosis? What would be
your further tests to confirm your diagnosis?
β-Thalassaemia major

 Chronic haemolysis due to excess α chains that precipitate in


the red cell forming Heinz bodies
 Bone marrow expands deforming bones
 Hepatomegaly & splenomegaly (extramedullary haemopoiesis)
 Possible gallstones, gout & jaundice with Chronic haemolysis
 Not usually detected until 6 months of age
 If not treated in childhood, classic thal develops within years
 ß Thal patients depend on transfusions, but excess iron from
transfusions may lead to cardiac and hepatic problems
 More iron is absorbed from the gut leading to Fe overload
 Largely overcome by the use of desferroxamine
 Cardiac failure can occur due to the constant high output of
blood required to maintain an adequate perfusion of tissues

Hb Low
Normal – Increased
RCC (Marrow compensating for ineffective
haemopoiesis)
MCH & MCV Low
Hypochromasia Markedly low
Anisocytosis Marked (Macrocytes, Microcytes)
Marked target cells,Tear Drops, Schistocytes
Poikilocytosis Acanthocytes, Howell Jolly Bodies, Target Cells
(post splenectomy)
Moderate polychromasia, nRBC (bone marrow
Immature Forms
response)
3. Fill in the genotype possibilities for Alpha thalassaemia:

One gene:
Two genes:
Alpha thal major/HbH disease:
Hydrops foetalis:

4. Young African-American women presented at the GP for regular health


check. Blood film morphology indicated moderate microcytosis,
hypochromia, anisocytosis and target cells. HbS was 35% and HbA
62%. What is your most likely diagnosis?
5. 46-year-old male recently moved from the Middle East to Alaska.
Fainted on a camping trip. On examination at the ED, patient was
finding it difficult to breathe and had splenomegaly. Blood film
morphology shows evidence of haemolysis. Antibody screening cells
negative at 37C. What should the transfusion scientist’s next move be?

6. What is the characteristic morphological feature of RBC you see in this


film. What is the provisional diagnosis? Explain its pathophysiology.

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