Pancytopenia Flashcards
What is the definition of pancytopenia?
Deficiency in blood cells in the blood (usually excludes lymphocytes)
What is the life span of neutrophils and platelets?
N = 7-8 hours P = 7-10 days
What problems involving reduced production can cause pancytopenia?
Bone marrow failure - primary or secondary
What is Fanconi’s syndrome?
An inherited marow failure syndrome
What are features of Fanconi’s?
Short stature Skin pigmented abnormalities Radial ray abnormalities Hypogenitalia Endocrinopathies GI defects Cardiovascular Renal Haematological
What skin changes can be seen in Fanconi’s?
Cafe au lait spots
What are the haematological issues in Fanconi’s?
Unable to correct inter-strand cross-links (DNA damage)
Macrocytosis followed by thrombocytopenia, then neutropenia
In Fanconi’s, what is the risk of bone marrow failure and leukaemia?
BM = 84% by 20 years L = 52% by 40 years
What are acquired primary bone marrow failure disorders?
An intrinsic marrow problem
- Aplastic anaemia (autoimmune attack against own haemopoietic stem cell
- Myelodysplastic syndromes (MDS)
- Acute leukaemia (white cell count can be variable)
Describe Myelodysplastic syndrome?
Dysplasia
Hypercellular marrow
increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)
Propensity for evolution into AML
Why can acute leukaemia cause pancytopenia?
Proliferation of abnormal cells (blasts) from leukaemia stem cells
Failure to differentiate or mature into normal cells
Prevent normal haemopoietic stem/progenitor development
Give examples of secondary bone marrow failure?
Drug induced (chemo/alcohol)
B12/folate deficiency (nuclear maturation)
Infiltrative - non-haemopoietic malignant infiltration, lymphoma
Viral/storage diseases (HIV)
What can cause increased destruction of blood cells?
Hyperslenism
What are the normal spleen values for a) splenic red cell mass, b) red cell transit and c) splenic platelet pool?
a) 5%
b) Fast
c) 20-40%
What are the hyperslenism values for a) splenic red cell mass, b) red cell transit and c) splenic platelet pool?
a) 40%
b) Slow
c) 90%
What are causes of hyperslenism?
Splenic congestion - Portal hypertension, Congestive cardiac failure
Systemic distress - RA (Felty’s)
Haematological - Splenic lymphoma
What 3 things predominantly make up pancytopenia?
Anaemia
Neutropenia
Thrombocytopenia
What are signs/symptoms of anaemia?
Fatigue
SOB
Cardiovascular compromise
What can occur in neutropenia?
Infections
What can occur in thrombocytopenia?
Bleeding
Purpura
Petechiae
What type of biopsy is used to examine bone marrow, and what needle is used?
Trephine biospy
Jamshidi needle
In pancytopenia, when is the marrow hypocellular?
In aplastic anaemia
In pancytopenia, when is the marrow hypercellular?
In myelodysplastic syndromes
B12/folate deficiency (maturation failure)
Hypersplenism
What supportive treatment can be offered for pancytopenia?
Red cell/platelet transfusion
ABx and prophylactic use of antibacterials, antifungals and antivirals
