Pancytopenia

Question 1

What is pancytopenia?

Answer 1

a decrease in all myeloid lineage cells

• erythrocytes
• platelets
• granulocytes

Question 2

Pancytopenia always means bone marrow failure. TRUE/FALSE?

Answer 2

FALSE
can be caused by other things
i.e. decreased production VS
increased destruction

Question 3

What inherited condition can cause bone marrow failure?

Answer 3

Fanconi’s anaemia

• problems with DNA repair => many congenital anomalies
• average age 7
• short stature, radial deformity, absent thumbs
• renal defects common

Question 4

What are the potential acquired causes of bone marrow failure?

Answer 4

• Idiopathic Aplastic anaemia
  => autoimmune response attacks haem stem cells
• Myelodysplastic syndromes (abnormal cells made then destroyed)
• acute leukaemia (lack of mature cells produced)

Question 5

What can cause secondary bone marrow failure?

Answer 5

Drugs => chemo/ chloramphenicol
B12/folate deficiency
Malignancy (metastatic or primary)
Viral e.g. HIV

Question 6

What are the causes of increased destruction?

Answer 6

• Hypersplenism
  => more blood cells trapped in spleen than in bloodstream
  => pancytopenia

Question 7

What are the potential causes of hypersplenism?

Answer 7

• congestion
  e. g. portal HT
• systemic disease
  => RA (Felty’s => large spleen and neutropenia)
• Haem disease
  => splenic lymphoma

Question 8

What are the clinical features of pancytopenia?

Answer 8

Anaemia (fatigue, SOB)
Neutropenia (infections of increased severity/duration)
thrombocytopenia (purpura/petechiae)

Question 9

What investigations are used to find a cause for pancytopenia?

Answer 9

FBC
Blood Film
B12 and folate
LFTs
Virology
Auto antibodies
Bone Marrow

Question 10

What specialised tests are used to investigate for inherited causes of pancytopenia?

Answer 10

cytogenetics

chromosome fragility testing

Question 11

Describe the cellularity of the bone marrow in aplastic anaemia?

Answer 11

HYPOcellular

Question 12

In what disorders can a HYPERcellular bone marrow be found?

Answer 12

• myelodysplastic
  => lots of abnormal cells being produced and removed, so bone marrow produces more in an attempt to keep up
• B12/folate deficiency
• hypersplenism (marrow attempts to counteract increased breakdown)

Question 13

What general treatment can be given in pancytopenia?

Answer 13

supportive
RBCs and platelet transfusion if req’d
antibiotic prophylaxis

Question 14

What specific treatment is given in primary bone marrow failure?

Answer 14

Malignancy - give chemo
Congenital - marrow transplant
Aplastic anaemia - immunosuppression

Question 15

What treatments can be used if a patient experiences secondary marrow failure?

Answer 15

• if due to drug reaction, then stop drug
• if viral cause, treat this (e.g. antiretroviral therapy for HIV)
• replace B12/folate if deficient
• if hypersplenic, treat cause or consider splenectomy