Haemolysis Flashcards
what is haemolysis
Premature red cell destruction
i.e. shortened red cell survival
why are red cells susceptible to damage
- need a biconcave shape to transit the circulation
- limited metabolic reserve and rely on glucose metabolism for energy (no mitochondria)
- Can’t generate new proteins once in the circulation (no nucleus)
what is compensated haemolysis
Increased red cell destruction compensated by increased red cell production
i.e. Hb Maintained
what is decompensated haemolysis also known as
Haemolytic anaemia
what is haemolytic anaemia
Increased rate of red cell destruction exceeding bone marrow capacity for red cell production
i.e. Hb Falls
what are consequences of haemolysis
Erythroid hyperplasia (increased bone marrow red cell production)
Excess red cell breakdown products eg billirubin (clinical features differ by aetiology and site of red cell breakdown)
what is the bone marrow response to haemolysis
1 - reticulocytosis
2 - erythroid hyperplasia [see a higher number of precursors of RBC in the bone marrow]
what special stain can be used to just see reticulocytes on a blood film
supravital stain
how can haemolytic anaemia be classified
extravascular
- RBC destroyed by spleen and liver
intravascular
- RBC destroyed within the circulation
what form of haemolytic anaemia is more common
extravascular
what are features of extravascular haemolytic anaemia
splenomegaly +/- hepatomegaly
Release of protoporphyrin
what does release of protoporphyrin cause
Unconjugated bilrubinaemia
> Jaundice and Gall stones
Urobilinogenuria
Normal products
what does it mean if bilirubin is unconjugated
pre hepatic cause
what is the pathophysiology of intravascular haemolysis
Red cells are destroyed in the circulation spilling their contents.
- Haemoglobinaemia (free Hb in circulation)
- Methaemalbuminaemia
- Haemoglobinuria: pink urine, turns black on letting the urine stand
- Haemosiderinuria
what is the big difference in symptoms between extra and intra vascular haemolysis
intravascular cause ABNORMAL products
- may be life threatening
what are causes of intravascular haemolysis
ABO incompatible blood transfusion
G6PD deficiency
Severe falciparum malaria
(Blackwater Fever)
PNH,PCH
what Ix are done first in haemolytic anaemia
FBC (+ BLOOD FILM) Reticulocyte count Serum unconjugated bilirubin Serum haptoglobins Urinary urobilinogen