pancytopaenia

Question 1

What is pancytopenia?

Answer 1

Low in all blood cell lineages

Tend to ignore lymphocytes in this case

Pancytopenia does not always mean bone marrow failure or malignancy

Question 2

What are the causes of pancytopenia?

Answer 2

Reduced production
-Bone marrow failure
*Acquired

1. Primary (intrinsic problem in haemopoietic SC or idiopathic immune defect)
   eg. Idiopathic aplastic anaemia (autoimmune attack against HSC)
   - bone marrow replaced by fat
   Acute leakaemia (total WBC can be high due to high circulating blasts, but patient would present with pancytopenia)
   Myelodysplastic syndromes (clonal HSC disorder characterised by dysplasia or unique genetic abnormalities; hypercellular marrow; increased apoptosis of progenitor and mature cells; propensity to develop into AML)
2. Secondary
   eg. Drug induced (chemotherapy, alcohol, azathioprine, methotrexate, chloramphenicol) - causes aplasia

B12/folate deficiency (nuclear maturation can affect all lineages) - causes hypercellular marrow

Infiltrative - non-haemopoietic malignant infiltration, lymphoma

Rare - viral/storage diseases

*Inherited (very rare, eg Fanconi’s anaemia - unable to correct inter-strand crosslinks => DNA damage)

Increased destruction
- Hypersplenism
Usually associated with splenomegaly
Splenic pool of RBCs and platelets increase drastically

Causes : Splenic congestion (portal hypertension), systemic disease (RA), Haematologicla causes (splenic lymphonma)

Question 3

Clinical features of pancytopenia

Answer 3

Pancytopenia = Anaemia + Neutropenia + Thrombocytopenia

Neutropenic patients can be susceptible to both Gram +ve and -ve bacteria (gram -ve is more severe and cause more damage in a short time span)

Bleeding & bruising

***Clinical features can come from CAUSES

Question 4

What is the cellularity of marrow in pancytopenia?

Answer 4

Variable depending on cause
- hypocellularin aplastic anaemia
Hypercellular in myelodysplastic syndromes, B12/folate def, hypersplenism

Question 5

How to treat pancytopaenia?

Answer 5

Supportive care - blood products, antibiotics
Treat neutropenic fever promptly based on local guideline without waiting for culture results

Specific - dependent on cuase