Pancytopenia Flashcards
What is pancytopenia?
A deficiency of blood cells of all lineages
Note*
Generally excludes lymphocytes
What are the two main things that cause pancytopenia?
Reduced production of cells
Increased destruction of cells
Give a common example of reduced cell production leading to pancytopenia?
Bone marrow failure - can be acquired or inherited
Give an example of an inherited bone marrow failure syndrome?
Fanconi’s anaemia
What are some clinical presentations of Fanconi’s anaemia?
Short stature
Skin pigment abnormalities
Hypogenitilia
Endocrinopathies
GI / CV defects
Skeletal abnormalities
Café au lait spots
What happens genetically in inherited bone marrow failure?
The body is unable to correct
inter-strand cross-links
DNA damage
Give 3 conditions that can lead to acquired primary bone marrow failure?
Idiopathic aplastic anaemia
Myelodysplastic syndromes
Acute leukaemia
What is idiopathic aplastic anaemia?
Autoimmune attack against haemopoietic stem cells
What are myelodysplastic syndromes?
Clonal haemopoietic stem cell disorders characterised by dysplasia or unique genetic abnormalities
What does myelodysplastic syndromes cause in a patient?
Increased apoptosis of progenitor and mature cells
What can myelodysplastic syndromes evolve into?
Acute myeloid leukaemia
Give 4 things that can cause secondary bone marrow failure?
B12 / folate deficiency
Drug induced - chemo, alcohol, azathioprine, methotrexate
Infection - viral, mycobacterial
Metastatic cancer - non-marrow malignancy
Give the most common reason of increased destruction of cells in patients with panytopenia?
Hypersplenism
Give 3 causes of hypersplenism?
Splenic congestion - portal hypertension
Systemic diseases - rheumatoid arthritis
Haematological diseases - splenic lymphoma
What are the common clinical features of pancytopenia?
Anaemia - fatigue, SOB
Neutropenia - infections
(gram negative causes sepsis)
Thrombocytopenia - bleeding
What investigations are used to establish the cause of pancytopenia?
History taking - family history
Clinical findings
FBC and blood film
Bone marrow examination
What is the marrow cellularity in aplastic anaemia?
Hypocellular
What is the marrow cellularity in myelodysplastic syndromes,
B12 / folate deficiency and hypersplenism?
Hypercellular
Give 2 supportive treatment options for patients with pancytopenia?
Red cell / platelet transfusions
Antibiotic prophylaxis / treatment
How is neutropenic fever treated?
Promptly following local unit antibiotic policy
DO NOT wait for microbiology results
How are malignant primary bone marrow disorders treated?
Chemotherapy
How are congenital primary bone marrow disorders treated?
Allogenic stem cell transplantation may be used
How is idiopathic aplastic anaemia treated?
Immunosuppression
How are drug-induced secondary bone marrow disorders treated?
Stop the drug
