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Haemolytic Anaemias: Dr. Suhair Abbas Ahmed
Haemolytic Anaemias: Dr. Suhair Abbas Ahmed
2- Site of defect:
• Intrinsic defect (intracorpuscular)-
structural or functional defect within the
red cell.
• Extrinsic defect (extracorpuscular)- an
abnormality in the red cell environment.
Classification of HA
3- Inherited or acquired:
• Inherited HA are usually caused by intrinsic defect.
• While acquired HA are caused by an extrinsic
defect.
• However there are some exceptions: Paroxysmal
nocturnal haemoglobinuria (PNH) which is an
acquired intrinsic defect, and severe
hereditaryG6PD enz deficiency which requires the
presence of an extrinsic trigger such as the
antimalarial drug for the intrinsic defect to
manifest.
Inherited & acquired HA
Hereditary HA Acquired HA
– Immune
• Membrane defects e.g
-Autoimmune eg AIHA
hereditary spherocytosis -Alloimmune e.g HDN,
• Metabolic defect e.g HTR
– Red cell fragmentation
G6PD deficiency. syndromes
• Haemoglobin defects – March
e.g sickle cell disease. haemoglobinaemia
– Infections
– Chemical and physical
agents.
– PNH
Approach to the diagnosis of
haemolytic anaemias.
Patient’s history
• Good history is essential to provide guidance for
the diagnosis of haemolytic disorders. The
following points should not be neglected:
• Family history--- hereditary conditions, mode of
inheritance.
• Ethnic origin--- G6PD deficiency is most common
in Mediterranean and Chinese populations.
• Past history--- NNJ may be indicative of
congenital conditions as HS or G6PD deficiency.
• Triggering events--- history of drugs, infections
Clinical features
• Pallor of the mucous membranes
• Mild fluctuating jaundice
• Splenomegaly
• Dark urine
• Pigment gall stones.
• Ulcers around the ankle
• Aplastic crisis may complicate viral infections.
• Growth retardation
• Hypertrophic skeletal changes
• Leg ulcers in
patients with severe
congenital
haemolytic
disorders. e.g sickle
cell anaemia
• Skeletal changes in
patients with
thalassaemia.
Laboratory findings
• The lab. Findings are divided into 3
groups:
1- Features of increased red cell
breakdown.
2- Features of increased red cell
production.
3- Damaged red cells.
Laboratory findings
1-Features of increased red cell
breakdown:
• Raised S.bilirubin, unconjugated and
bound to albumin.
• Increased urine urobilinogen.
• Increased faecal stercobilinogen.
• Absent S.haptoglobins (saturated with
Hb and removed by the RE cells).
Laboratory findings
2-Features of increased red cell
production:
• Reticulocytosis
• Bone marrow erythroid hyperplasia.
Laboratory findings
• 3-Damaged red cells:
• Morphology– microspherocytes,
elliptocytes, fragments, etc.,….
• Special tests: Osmotic fragility,
autohaemolysis…..
• Red cell survival is shortened; this is
best shown by 51Cr labelling with study
of the sites of destruction.
• Reticulocytosis is a
feature of increased
red cell production.
• New methylene blue
is used to stain the
reticulocytes
• Fragmented cells,
and bitten cells are
sings of damaged
cells occurring in
haemolysis
Intravascular haemolysis
Intravascular haemolysis
• Free Hb will be released from damaged red cells.
• This free Hb will rapidly saturates plasma
haptoglobins. The complex will be removed by
the liver.
• The excess free Hb is filtered by the glomerulus,
and free Hb will enter the urine, as iron is
released, the renal tubules become loaded with
haemosiderin.
• Methaemalbumin and haemopexin are also found
in the process of IV haemolysis.
• The process of
intravascular
haemolysis
• Liberation of free Hb
• Filtered through the
kidney
• Appear in urine as
haemoglobinuria
Lab features of intravascular
haemolysis