Page 91 - Essential Haematology

Chapter 6 Haemolytic anaemias / 77

also formed from the process of intravascular

Table 6.2 Causes of intravascular
haemolysis. haemolysis.
The main laboratory features of intravascular

haemolysis are:
Mismatched blood transfusion (usually ABO)
G6PD defi ciency with oxidant stress 1 Haemoglobinaemia and haemoglobinuria;
2 Haemosiderinuria (iron storage protein in the
Red cell fragmentation syndromes
spun deposit of urine (Fig. 6.3 b));
Some autoimmune haemolytic anaemias 3 Methaemalbuminaemia (detected spectrophoto-
Some drug - and infection - induced haemolytic metrically by Schumm ’ s test).
anaemias
Paroxysmal nocturnal haemoglobinuria Hereditary h aemolytic a naemias
March haemoglobinuria
Membrane d efects
Unstable haemoglobin
Hereditary s pherocytosis
G6PD, glucose - 6 - phosphate dehydrogenase. Hereditary spherocytosis (HS) is the most common
hereditary haemolytic anaemia in northern
Europeans.

(a)

Figure 6.3 (a) Progressive urine

samples in an acute episode of
intravascular haemolysis showing
haemoglobinuria of decreasing
severity. (b) Prussian blue -
positive deposits of haemosiderin
in a urine spun deposit (Perls ’
stain). (b)