Pancytopenia

Question 1

What is pancytopenia?

Answer 1

Deficiency of all blood cell lineages- anaemia, neutropenia, thrombocytopenia

Not a diagnosis but giveaway sign for other conditions

Question 2

What are the 2 classes of aetiology of pancytopenia?

Answer 2

Reduced production
Increased destruction

Question 3

Inherited causes of reduced production

Answer 3

Inherited syndrome-

defects in DNA repair/ribosomes/telomeres -> impaired haemopoesis, congenital abnormalities and cancer predisposition

Eg FANCONI’S ANAEMIA

unable to correct inter-strand cross-links -> DNA damage

Question 4

Acquired primary causes of reduced production

Answer 4

Primary- no obvious cause

Idiopathic aplasic anaemia - autoreactive T-cells target haemapoeisis
Myelodysplastic syndromes - increased apoptosis of progenitor and mature cells, propensity for evolution into AML- not all of these patients will get Pancytopenia
Acute leukaemia (WCC can be variable) - proliferation of abnormal cells from LSC, failure to differentiate into mature or normal cells, prevent normal HSC development

Question 5

Acquired secondary causes of acquired reduced production

Answer 5

-drug induced e.g. chemotherapy, alcohol, azathioprine, metotrexate, chlorampenicol - causes aplasia
-B12/folate deficiency - nuclear maturation can affect all lineages
-Infiltrative - non-haemopoietic malignant infiltration, lymphoma
-Misc. - viral (e.g. HIV), storage diseases

Question 6

What causes increased destruction?

Answer 6

Hypersplenism

Splenomegaly causes increased destruction when rate exceeds bone marrow capacity

Causes of hypersplenism-
Splenic congestion eg portal hypertension
Systemic disease eg RA
Haematological conditions eg splenic lymphoma

Question 7

What is the life span of:
RBCs
WBCs
Platelets

Answer 7

120 days
7-8 hours
7-10 days

Question 8

How does Pancytopenia present?

Answer 8

Pancytopenia is triad of- anaemia, neutropenia, thrombocytopenia

Anaemia symptoms- SOB, fatigue
Neutropenia symptoms- infection susceptibility
Thrombocytopenia symptoms- easy bleeding, purpura and petechiae

Plus influence from underlying cause

Question 9

How is pancytopenia investigated?

Answer 9

Bloods- FBC and film

Routine tests influenced by history and clinical findings

Bone marrow exam
Specialist tests eg cytogenetics for fanconi

Question 10

Management for Pancytopenia

Answer 10

Supportive-
Red and platelet transfusions
Prophylaxis when needed
Treat neutropenic fever empirically

Specific- Dependent on cause

Primary bone marrow disorder

• Malignancy - consider chemotherapy
• Congenital - consider bone marrow transplantation
• Idiopathic aplastic anaemia- immunosuppression

Secondary bone marrow disorder

• Drug reaction - STOP
• Viral - e.g. treat HIV
• Replace B12/folate

Hypersplenism

• Treat cause if possible
• Consider splenectomy (not appropriate in all cases)