haemolytic anaemia

admittedly I still don't really get it.

a definition of sorts

  • shortened RBC lifespan

clinical presentation

associated disease states etc

the causes are many: classify or die

  • intravascular
    • immune-mediated vs non-immune-mediated?
  • extravascular
    • intrinsic to RBC vs extrinsic?
Intravascular Extravascular
Immune

- ABO or Rhesus incompatibility
- Haemolytic disease of the newborn (HDN)
- Transfusion mismatch
- IgG-mediated “warm” autoimmune haemolytic anaemia (AIHA)
- drugs e.g. methyldopa, penicillin, cephalosporins, erythromycin, probenecid, paracetamol and ibuprofen
- autoimmune diseases e.g. antiphospholipid syndrome, SLE, Rheumatoid arthritis
- lymphoproliferative diseases e.g. lymphoma, CLL
- idiopathic
- “cold” AIHA = complement-mediated

Non-immune

- mechanical e.g. cardiac peri-prosthetic or peri-valvular leaks, intravascular devices (e.g. TIPS), extracorporeal circuits (e.g. ECMO, RRT)
- infection
- e.g. severe sepsis, malaria (e.g. blackwater fever), Clostridium perfringens (Necrotising fasciitis), babeosis
- hypotonic solutions and drowning
- toxins e.g. heavy metals, oxidants (e.g. causes of Methaemoglobinaemia), sodium chlorate

- Microangiopathic haemolytic anaemia (MAHA)
- e.g. envenoming (e.g. australian elapid), malignancy, malignant hypertension, sisseminated intravascular coagulation (DIC), pre-eclampsia/eclampsia
- Thrombotic microangiopathy (MAHA, thrombosis and thrombocytopenia), e.g. HUS, TTP and HELLP
- Trauma, burns
- Liver disease
- e.g.  Zieve’s syndrome due to alcoholic cirrhosis, Wilson’s disease
Intrinsic

- enzyme abnormalities
- G6PD deficiency
- pyruvate kinase deficiency
- membrane abnormalities
- hereditary spherocytosis
- hereditary eliptocytosis
- paroxysmal nocturnal hemoglobinuria
- hemoglobinopathies
- Sickle cell disease
- thalassemia

Extrinsic
hypersplenism

- portal hypertension from liver disease
- myeloproliferative diseases
- tropical splenomegaly syndrome

or: warm vs cold (...)

diagnostic investigations

  • Hb (expect ↓)
  • reticulocytes (expect ↑)
  • LDH (expect ↑)
  • LFTs (expect ↑ indirect/unconjugated bilirubin)
  • Intravascular: haemoglobinuria, haemosiderinuria, ↓ haptoglobin