HAEMOLYTIC

ANEMIA
Dr. Sookun Rajeev K
(MD)
Dept of General Medicine
Anna Medical College
 DEFINITION:
•Hemolytic anemias are caused by decreased red
blood cell survival from increased destruction of
the cells.

•The destruction may be either in the blood

vessels (intravascular) or outside the vessels
(extravascular),which generally means inside the
spleen.
CLASSIFICATION
1. Congenital / Hereditary
2. Acquired
 ETIOLOGY
The hemolytic anemias may either be:
CHRONIC as in sickle-cell disease, paroxysmal
nocturnal hemoglobinuria and hereditary
spherocytosis
OR
ACUTE such as in drug induced hemolysis,
autoimmune hemolysis or glucose 6-phosphate
dehydrogenase deficiency.
 HEMOLYTIC ANEMIA
Causes
•INTRACORPUSCULAR HEMOLYSIS
Membrane Abnormalities
Metabolic Abnormalities
Hemoglobinopathies

•EXTRACORPUSCULAR HEMOLYSIS
Nonimmune
Immune
 HEMOLYTIC ANEMIA
Membrane Defects
•Microskeletal defects
• Hereditary spherocytosis

•Membrane permeability defects

• Hereditary stomatocytosis

•Increased sensitivity to complement

• Paroxysmal nocturnal hemoglobinuria
 CLINICAL PRESENTATION
The usual symptoms of anemia are present based on the
severity of the disease, not necessarily the etiology.

Fatigue and weakness occur with mild disease.

Dyspnea and later confusion with more severe disease.

Hemolysis is often associated with jaundice and dark urine

as well.
 CLINICAL PRESENTATION
Fever
Chills
Chest pain
Tachycardia
Backache may occur if the intravascular
hemolysis is particularly rapid.
 DIAGNOSIS
•Hemolytic anemias generally have a normal MCV
but the MCV may be slightly elevated because
reticulocytes are somewhat larger than molder
cells.
•The reticulocyte count is elevated.
•The Lactate Dehydrogenase (LDH) and indirect
bilirubin are elevated. Bilirubin levels above four
are unusual with hemolysis alone.
 DIAGNOSIS
• The peripheral smear shows fragmented cells and the
haptoglobin may be low with intravascular hemolysis.
The haptoglobin level is often unreliable.
• Hemoglobin may be present in the urine when
intravascular hemolysis is sudden and severe because
free hemoglobin spills into the urine.
• There should not be bilirubin in the urine because
indirect bilirubin is bound to albumin and should not
filter at the glomerulus.
 TREATMENT
• Transfusion is needed as in all forms of anemia when the
hematocrit becomes low.

• Hydration is, in general, useful to help prevent toxicity to the

kidney tubule from the free hemoglobin.

• Folic Acid 5mg weekly, prophylaxis life long

• Splenectomy

• Blood transfusion in Acute, severe hemolytic crisis