Pancytopenia Flashcards
What is the lifespan of red cells?
120 days
Life span of neutrophils?
7-8 hours
What is the lifespan of platelets?
7-10 days
Clinical presentation of Fanconi’s anaemia
Short stature Skin pigment abnormalities Radial rayabnormalities Hypogenitilia Endocrinopathies GI defects Cardiovascular Renal Haematological
Pathophysiology of Fanconi’s anaemia
Inherited marow failure
Unableto correct inter-strand cross-links (DNA damage)
What is aplastic anaemia?
Autoimmune attack against haemopoietic stem cell
Pathophysiology of aplastic anaemia
CD8 T cells produce IFN-gamma & TNF-alpha which means stem cell compartment is significantly reduced
What is myelodysplastic syndrome?
Dysplasia leading to hypercellular marrow
Increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)
What can myelodysplasitc evolve into?
AML (passenger mutations can acquire driver mutations)
Why can acute leukaemia cause pancytopenia?
Proliferation of abnormal cells from leukaemic stem cells
Failure to differentiate or mature into normal cells
Prevent normal haemopoietic stem development by hijacking the haemopoietic niche and marrow microenvironment
What drugs can cause secondary bone marrow failure?
Chemotherapy
Chloramphenicol
Alcohol
Causes of secondary bone marrow failure?
Drugs B12/folate deficiency Infections Storage disorders (Gauchers) Metastic cancer (non-marrow malignancy)
Causes of Hypersplenism
Splenic congestion (portal hypertension, CCF) Systemic diseases (Rheumatoid Arthritis - Felty's) Haematological diseases (Splenic lymphoma)
Signs & symptoms of pancytopenia
Anaemia
Neutropenia (infections)
Thrombocytopenia (bleeding)
How should neutropenic fever be treated in pancytopenia?
Promptly based on local policy without waiting for microbiology results
