Pancytopenia Flashcards
What is pancytopenia?
A deficiency of blood cells of all lineages (however generally excludes lymphocytes0 - not a diagnosis, and does not always mean bone marrow failure or malignancy.
What are the 2 main reasons for pancytopenia occurring?
Reduced production - bone marrow failure
Increased destruction - hypersplenism
What are some inherited syndrome features of reduced blood cell production?
Characterised by impaired haemopoiesis, congenital abnormalities and cancer predisposition.
Arise due to defects in DNA repair/ribosomes/telomeres.
Example: patients with Fanconi’s anaemia are unable to correct inter-strand cross-links (DNA damage).
What are some primary acquired features of reduced blood cell production?
Primary - no obvious cause, usually stem cell defect:
- Idiopathic aplastic anaemia - autoreactive T cells target haemopoiesis.
- Myelodysplastic syndromes - increased apoptosis of progenitor and mature cells, possibility of evolving into AML.
- Acute leukaemia (WCC can be variable) - proliferation of abnormal cells from LSC, failure to differentiate into mature or normal cells.
What are some secondary acquired features of reduced blood cell production?
Secondary:
Drug induced e.g. chemotherapy, alcohol, azathioprine, methotrexate.
B12/folate deficiency - nuclear maturation can affect all lineages.
Infiltrative - non-hemopoietic malignant infiltration, lymphoma.
Miscellaneous - viral (e.g. HIV), storage diseases.
How does hypersplenism occur in pancytopenia?
Pancytopenia can be caused by increased splenic pool or increased destruction that exceeds bone marrow capacity, usually associated with a significantly large spleen.
What are causes of hypersplenism?
Splenic congestion e.g. portal hypertension.
Systemic diseases e.g. rheumatoid arthritis
Haematological diseases e.g. splenic lymphoma
Clinical features of pancytopenia?
Anaemia - fatigue, shortness of breath, cardiovascular compromise.
Neutropenia - increased severity and duration of infections, opportunistic infections.
Thrombocytopenia - bleeding (purpura, petechiae, “wet” bleeds).
Presentation can also be influenced by underlying cause
Blood test investigations for pancytopenia?
FBC, blood film
Additional routine tests guided by history (including family history), clinical findings and initial blood results.
Supportive management for pancytopenia?
Transfusions - red cell transfusions, platelet transfusions, (neutrophil transfusions are NOT routine).
Antibiotic prophylaxis/treatment where appropriate.
Treat neutropenic fever promptly based on local unit antibiotic policy without waiting for microbiology results.
Primary bone marrow disorder management in pancytopenia?
Malignancy - consider chemotherapy
Congenital - consider bone marrow transplantation
Idiopathic aplastic anaemia - immunosuppression
Secondary bone marrow disorder management in pancytopenia?
Drug reaction - STOP
Viral - e.g. treat HIV
Replace B12/folate
Hypersplenism management in pancytopenia?
Treat cause if possible
Consider splenectomy (not appropriate in all cases).
What is bone marrow cellularity?
Bone marrow cellularity refers to the density or concentration of cells within the bone marrow.
It indicates how much of the marrow space is occupied by cells, including various types such as red blood cells, white blood cells, and platelets.
This measurement is important in evaluating bone marrow health and function, as changes in cellularity can indicate different medical conditions or responses to treatments.
Cause of hypocellularity in bone marrow?
Aplastic anaemia
