Pancytopenia Flashcards
Define pancytopenia?
a deficiency of blood cells of all lineages (but generally excludes lymphocytes)
it is more a reduction in neutrophils than other white cells
Pancytopenia can be due to _______ or _______
reduced production (bone marrow failure) increased destruction
What is an inherited cause of bone marrow failure?
Faconi’s anaemia
Acquired bone marrow failure problems can be _______ or ________
primary (issue with bone marrow) or secondary (other disorder affecting the bone marrow)
Give some examples of acquired primary bone marrow failure problems?
idiopathic aplastic anaemia
myelodysplastic syndromes
acute leukaemia
Give some examples of acquired secondary bone marrow failure problems?
storage disorders metastatic cancer infections vitamin deficiency e.g. B12 or folate drug induced aplasia
What is the main cause of increased destruction?
hypersplenism (any cause of splenomegaly)
e.g. portal hypertension, RA, splenic lymphoma
Describe Faconi’s anaemia?
rare
inherited disorder
causes bone marrow failure (aplastic anaemia), congenital anomalies and cancer pre disposition
anomalies: skeletal, cafe au lair, endocrine issues, short stature, GI, CVS and renal issues
What is idiopathic aplastic anaemia?
auto-immune attack against haemopoietic cells
hypocellularity/ aplasia of the bone marrow
What are myelodysplastic syndromes?
disordered cell development in the marrow resulting in a hyper cellular marrow
the dysplasia causes ineffective haemopoiesis and there is increased apoptosis of progenitor and mature cells
What do myelodysplastic have a significant risk of?
development into AML
What drugs can induce marrow failure? What does the marrow look like?
e.g. chemo, alcohol, azathioprine, methotrexate, chloramphenicol
causes aplasia/ hypocellular marrow
How does B12/ folate deficiency cause pancytopenia? What does the marrow look like?
defects in nuclear maturation can affect all the cell lineages
causes a hypercellular marrow
What are the clinical features of pancytopenia?
symptoms of pancytopenia > anaemia, neutropenia, thrombocytopenia
symptoms of the cause
Marrow is hypocellular in
aplastic anaemia (faconis, drug induced and idiopathic autoimmune)
