Pancytopenia Flashcards
What are the features of Fanconi’s anaemia?
Short stature
Skin pigment abnormalities
Radial ray abnormalities
Hypogenitilia
Endocrinopathies
GI defects
Cardiovascular
Renal
Haematological
What is the pathology in Fanconi’s anaemia?
Unable to correct inter-strand cross-links (DNA damage)
What haematological cell abnormalities occur in Fanconi’s anaemia?
Macrocytosis followed by thrombocytopenia, then neutropenia
What is the risk of developing bone marrow aplasia with Fanconi’s anaemia?
84% by 20 years
What is the risk of developing leukaemia with Fanconi’s anaemia?
52% by 40
What are some of the causes of acquired primary bone marrow failure?
Intrinsic bone marrow problems:
- aplastic anaemia
- myelodysplastic disorders
- acute leukaemia
What are some of the causes of secondary bone marrow failure?
Drugs e.g. chemotherapy, chloramphenicol
B12 or folate deficiency
Malignant: non-haemopoietic infiltration, lymphoma
What is aplastic anaemia?
Autoimmune attack against the haemopoetic stem cell
What can cause increased destruction of haematological cells causing pancytopenia?
Hypersplenism
Autoimmune
What is the difference in the splenic pool in a normal spleen and in hypersplenism?
Increased splenic pool
What is pancytopenia?
Anaemia, thrombocytopenia and neutropenia
How will the marrow cellularity appear in aplastic anaemia?
Hypocellular
What things will cause a hypercellular picture in bone marrow?
Myelodysplastic syndromes
B12/folate deficiency (maturation ‘failure’)
Hypersplenism
How is neutropenic sepsis managed?
Barrier nursing
GCSF
Piperacillin
Tazobactam
Gentamicin if severe
What is CD20 a marker of?
B cells (non-Hodgkins)