Hemolytic Anemia Classification, Investigations and Treatment

Hemolytic Anemia

Hemolytic anemia is a reduction in a number of circulating red cells from their premature destruction.

Hemolytic Anemia Etiological Classification

1. Acquired or Extracorpuscular

1. Immunohemolytic anemia:
   • Autoimmune hemolytic anemia
   • Warm antibody autoimmune hemolytic anemia
   • Cold antibody autoimmune hemolytic anemia
   • Drug-induced immune hemolytic anemia
   • Iso immune hemolytic anemia.
2. Mechanical trauma: Micro­angiopathic hemolytic anemia
3. Direct toxic effect: Malaria, bacteria, infection, and other agents
4. Acquired red cell membrane abnormalities: Paroxysmal nocturnal hemoglobinuria.
5. Splenomegaly.

2. Hereditary or Intracorpuscular

1. Abnormalities of red cell membrane:
   • Hereditary spherocytosis
   • Hereditary elliptocytosis
   • Hereditary stomatocytosis.
2. Disorders of red cell interior:
   • Red cell enzyme defect:
   • Defects in HMP shunt: G6PD defiiency
   • Defects in the glycolytic pathway: Pyruvate kinase defiiency.
3. Disorders of hemoglobin:
   • Structurally abnormal hemoglobin, sickle syndrome, and other hemoglobinopathies.
   • Reduced globin chain synthesis: Thalassemias.

Hemolytic Anemia Clinical Features

• There is the presence of high fever, toxemia, marked*prostration, shock, and hemoglobinuria
• Acute renal failure may develop
• The chronic form includes jaundice varying from mild to severe form, mongoloid faces, splenomegaly, chronic leg ulcers, and pigment stones in the gallbladder.

Investigations or diagnosis for Hemolytic anemia

Test of Increased red cell Breakdown

• Blood film shows normocytic normochromic or dimorphic anemia.
• Unconjugated serum bilirubin is raised
• Urine urobilinogen is raised but bilirubinuria is absent
• Serum hemoglobin is reduced
• Plasma LDH is increased
• There is evidence of intravascular hemolysis i.e.hemoglobinaemia, hemoglobinuria, methemoglobinemia,hemosiderinuria.

Test Of Damage To Red Cells

• Blood film shows microspherocytes and fragmented RBCs.
• Osmotic fragility is increased.
• Electrophoresis test is done for abnormal hemoglobin
• Estimation of Hemoglobin A2 should be done
• Estimation of Hemoglobin F should be done
• Test for sickling is done
• Screening test for G6PD defiiency is done

Test For Shortened Red Cell Survivor The chromium labeled method shows a short red cell life span.

Treatment Of Hemolytic Anemia

• Patients with compensated hemolytic processes need no treatment.
• Mainly management is general and specific.
• Folic acid 5mg is given routinely and lifelong in patients with inherited hemolytic disorders
• Patients having hereditary spherocytosis may undergo splenectomy if they have moderate to severe disease or have experienced episodes of hemolytic crisis or gall stones.

Classification Of Hemolytic Anemia

Hemolytic anemia is a reduction in a number of circulating red cells from their premature destruction.

Hemolytic Anemia Etiological Classification

1. Acquired or Extracorpuscular

1. Immunohemolytic anemia:
   • Autoimmune hemolytic anemia
   • Warm antibody autoimmune hemolytic anemia
   • Cold antibody autoimmune hemolytic anemia
   • Drug-induced immune hemolytic anemia
   • Iso immune hemolytic anemia.
2. Mechanical trauma: Micro­angiopathic hemolytic anemia
3. Direct toxic effects: Malaria, bacteria, infection, and other agents
4. Acquired red cell membrane abnormalities: Paroxysmal nocturnal hemoglobinuria.
5. Splenomegaly.

2. Hereditary or Intracorpuscular

1. Abnormalities of cell membrane:
   • Hereditary spherocytosis
   • Hereditary elliptocytosis
   • Hereditary stomatocytosis.
2. Disorders of cell interior:
   • Red cell enzyme defect:
   • Defects in HMP shunt: G6PD defiiency
   • Defects in the glycolytic pathway: Pyruvate kinase defiiency.
3. Disorders of hemoglobin:
   • Structurally abnormal hemoglobin, sickle syndrome, and other hemoglobinopathies.
   • Reduced globin chain synthesis: Thalassemias.

Hemolytic Anemia Clinical Features

There is the presence of high fever, toxemia, marked prostration, shock, and hemoglobinuria Acute renal failure may develop

The chronic form includes jaundice varying from mild to severe form, mongoloid faces, splenomegaly, chronic leg ulcers, and pigment stones in the gallbladder.

Investigations or diagnosis for Hemolytic anemia

Test of Increased red cell Breakdown

• Blood film shows normocytic normochromic or dimorphic anemia.
• Unconjugated serum bilirubin is raised
• Urine urobilinogen is raised but bilirubinuria is absent
• Serum hemoglobin is reduced
• Plasma LDH is increased
• There is evidence of intravascular hemolysis i.e.hemoglobinaemia, hemoglobinuria, methemoglobinemia,hemosiderinuria.

Test Of Increased Red Cell Production Reticulocyte count increased

• Blood film shows macrocytosis, polychromasia and the presence of normoblasts
• Bone marrow examination shows erythroid hyperplasia with raised iron storage.
• X-ray of bones shows the expansion of marrow space in bones such as the skull.