Pancytopenia

Question 1

Definition of pancytopenia?

Answer 1

A deficiency of blood cells of all lineages

Question 2

Causes of pancytopenia due to low production?

Answer 2

Bone marrow failure (eg Fanconi’s anaemia)
- Inherited syndromes due to defects in DNA repair/ribosomes

Acquired primary(Usually stem cell defect) or secondary

Question 3

Features of Fanconi’s anaemia

Answer 3

Short stature
Skin pigment abnormalities (eg cafe au lait)
Radial ray abnormalities
Hypogenitalia
GI
Cardio
Renal
Haem

Question 4

Examples of acquired primary bone marrow failure

Answer 4

Aplastic anaemia (Autoimmune against haemopoietic stem cells)

Myelodysplastic syndromes

Acute leukaemia

Question 5

Pathogenesis of aplastic anaemia?

Answer 5

Auto reactive t cells (eg IFN gamma and TNF alpha) attack haemopoietic stem cells

Question 6

Pathology behind myelodysplastic syndrome

Answer 6

Increase apoptosis of progenitor and mature cells

Hypercellular marrow

Question 7

Why can acute leukaemia cause pancytopenia?

Answer 7

Prolif of abnormal cells from leukaemic stem cells

Failure to differentiate or mature

prevent normal haemopoietic stem cell development by hijacking marrow environment

Question 8

Causes of secondary bone marrow failure?

Answer 8

Drug induced–>Aplasia
B12/folate deficient
Infiltrative
Viral/misc

Question 9

Increased destruction of blood cells cause?

Answer 9

Hypersplenism

Question 10

Causes of hypersplenism?

Answer 10

Congestion
Systemic (eg feltys-RA)
Splenic lymphoma (Splenic lymphoma)

Question 11

Signs of pancytopenia?

Answer 11

Anaemia
Neutropenia
Thrombocytopenia

Question 12

Symps associated w/ anaemia?

Answer 12

Fatigue
SOB
CV compromise

Question 13

Symps associated w/ neutropenia?

Answer 13

Infections that are recurrent, severe and last long time

Question 14

Symptoms of thrombocytopenia?

Answer 14

Bleeding-Purpura and petechiae

Question 15

Supportive treatment for pancytopenia?

Answer 15

Red cell/platelet transfusion

Abx