Pancytopenia

Question 1

What is pancytopenia

Answer 1

• A deficiency of blood cells of all lineages
• (but generally excludes lymphocytes)
• anaemia, leukaemia, thrombocytopenia, neutropenia
• is not a diagnosis
• doesn’t always mean marrow failure/malignancy

Question 2

Pancytopenia aetiology

Answer 2

Inherited bone marrow failure
- fanconi’s anaemia (unable to correct DNA damage)

Primary (acquired) bone marrow failure
- Idiopathic aplastic anaemia
- Myelodysplastic syndromes
- Acute leukaemia

Secondary (acquired) bone marrow failure
- Drug induced e.g. methotrexate, chemo, alcohol
- B12/folate deficiency
- Infiltrative non haemopoietic malignancy or lymphoma
- Viral e.g. HIV
- Storage disorders

Increased destruction - hypersplenism
- Splenic congestion e.g. portal hypertension
- Systemic diseases e.g. rheumatoid arthritis
- Haematological diseases e.g. splenic lymphoma

Question 3

What are the three characteristics of inherited bone marrow syndromes

Answer 3

• Impaired haemopoiesis
• Congenital anomalies
• Cancer pre-disposition

Question 4

Primary vs secondary acquired bone marrow failure

Question 5

What is idiopathic aplastic anaemia

Answer 5

Autoimmune T cell attack against HSCs & progenitors

Question 6

What is myelodysplastic syndrome

Answer 6

Clonal HSC disorder
characterised by dysplasia &/ unique genetic abnormalities
can cause isolated macrocytic anaemia or pancytopenia (with dysplasia of all lineages)

Question 7

Myelodysplastic syndrome bone marrow biopsy findings

Answer 7

Hypercellular marrow
Increased apoptosis of progenitor & mature cells
=> Ineffective haemopoiesis

Question 8

Are all patients with Myelodysplastic syndrome pancytopenic

Answer 8

No - Isolated macrocytic anaemia is more common

Question 9

What does myelodysplastic syndrome predispose to?

Answer 9

Acute myeloid leukaemia

Question 10

What drugs can cause pancytopenia & why is this

Question 11

What deficiency can cause pancytopenia & why

Question 12

Why can acute leukaemia cause pancytopenia

Question 13

Does the size of the spleen correlate with the severity of pancytopenia

Answer 13

Not necessarily

Question 14

Pancytopenia clinical presentation

Answer 14

• Anaemia - fatigue, SOB, CV compromise
• Neutropenia - increased severity and duration of infections, opportunistic infections
• Thrombocytopenia - bleeding (purpura, petechiae, ‘wet’ bleeds)
• Presentation can also be influenced by underlying cause

Question 15

What cause hypocellular marrow, pancytopenia

Answer 15

Aplastic anaemia

Question 16

What causes hypercellular marrow, pancytopenia

Answer 16

Myelodysplastic syndromes (increased apoptosis),
B12/folate deficiency (increased apoptosis),
hypersplenism

Question 17

Aetiology focused pancytopenia investigations

Question 18

Pancytopenia supportive management

Answer 18

Red cell transfusions
Platelet transfusions
Antibiotics prophylaxis (antibacterials (gram -Ve), antifungals)
Treat neutropenic promptly (don’t wait for results)