Aplastic Anaemia

Definition and causes

  • Pancytopaenia with bone marrow hypocellularity
  • Acquired
    • Cytotoxic chemotherapy
    • Radiation
    • Seronegative hepatitis (most common) / HIV / EBV / PB19
    • Graft v. host
    • Paroxysmal nocturnal haemoglobinuria
    • Immune disease
    • Hyperimmunoglobulinaemia
    • Benzene and other chemicals
    • Drugs
  • Genetic
    • Fanconi’s anaemia
    • Dyskeratosis congenita

Presentation

  • Pancytopaenia
  • Striking limitation of disease to haemtological system
    • BLeeding
    • Anaema
    • Infection is uncommon as first presentation
  • Weight loss / systemic symptoms implies another idsease process

Labs

  • Bloods
    • Large RBCs, lack of platelets and neutrophils
    • MCV increased
    • Few retics
    • Lymphocytes may be normal or low
    • Immature myeloid forms implies MDS or AML
  • Marrow
    •  Readily aspirable
    • Haemtopoietic cells <25% space
    • Lots of fat
  • Others
    • In kids, diepoxybutane or mitomycin C to exclude Fanconi’s anaemia
    • Telomere length > telomerase or sheltrin mutatin
    • Chromosome studies should be negative (e.g. Downs etc. implies MDS)
    • Flow cytometry (CD59-, CD55- > PNH)
    • Viral screens
    • Imaging

Treatment

  • HSC transplant
  • ATG + cyclosporin
  • Supportive transfusion
Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s