Haemolytic anaemia is caused by the destruction of red blood cells at an accelerated pace beyond the capacity of the bone marrow to compensate. Usually, a normocytic picture is present.
Haemolytic anaemia
| Association | Coombs | Other tests | Treatment | |
|---|---|---|---|---|
| Warm AIHA | Autoimmune condition, Lymphoma | Positive | – | Corticosteroids |
| Cold AIHA | Infection and exposure to cold | Positive | Cold agglutinin titer | Avoid cold |
| Drug-induced AIHA | Drug exposure (cephalosporin #1) | Variable | – | Remove offending drug |
| Hereditary spherocytosis | Splenomegaly and FHx | Negative | Spherocytes in PBF | Splenectomy if severe |
| Paroxysmal nocturnal haemoglobinuria | Cola-coloured urine in the morning +/- pancytopenia, thrombocytopenia | Negative | Flow cytometry for CD55/59 | Eculizumab |
| G6PD deficiency | Male, Intermittent hemolysis following oxidant | Negative | Heinz bodies and bite cells in PBF | Avoid oxidants |
Classification of hemolytic anemia
| Classification | Examples |
|---|---|
| Intrinsic hemolytic anemia | Membranopathy, Enzymopathy, Hemoglobinopathy |
| Extrinsic hemolytic anemia | Autoimmune and alloimmune hemolysis, Mechanical destruction, Toxin-mediated destruction, Hypersplenism |
Mechanism of RBC destruction
| Mechanism | Description |
|---|---|
| Intravascular hemolysis | Hemolysis in blood vessels |
| Extravascular hemolysis | Hemolysis that occurs in the spleen or liver |
Coombs positive vs Coombs negative hemolytic anaemia
| Coombs | Examples |
|---|---|
| Coombs (+) | Warm and Cold autoimmune hemolytic anemia, Drug-induced hemolytic anemia |
| Coombs (-) | Hereditary spherocytosis, Paroxysmal Hemoglobinuria, Glucose-6-Phosphate deficiency, Sickle cell disease |
- Signs and symptoms
- Symptoms of anaemia
- Splenomegaly – due to trapping and breakdown of RBCs in the spleen
- Jaundice and pruritus – due to degradation of hemoglobin into bilirubin)
- Gallstones – due to hyperbilirubinemia
- Hemosiderinuria – due to destruction of RBCs
- Constitutional symptoms in acquired hemolytic anaemia
- Fever
- Weakness
- Chills
- Investigations
- Complete blood count
- Decreased RBC count
- Decreased Hb
- Decreased HCT
- Normal MCV
- Elevated WBC
- Decreased Platelets in MAHA
- Reticulocyte count
- Elevated reticulocyte count
- Peripheral blood film
- Reticulocytosis
- Intravascular hemolysis
- Heinz bodies (G6PD deficiency)
- Schistocytes (MAHA and macroangiopathic haemolytic anemia)
- Intracellular organisms (malaria, babesiosis, bartonellosis)
- Extravascular hemolysis
- Spherocytes (Hereditary spherocytosis and immune-mediated hemolysis)
- RBC agglutination in CAD
- Sickle cells in sickle cell disease
- Target cells in sickle cell disease, thalassemia, and HbC disease
- Teardrop cells in thalassemia
- Hb Crystals in HbC disease
- Smudge cells (Gumprecht shadows) in CLL
- Serum studies
- Decreased haptoglobin
- Increased LDH
- Elevated indirect bilirubin
- Urinalysis
- Haemoglobinuria
- Hemosiderinuria
- Urobilinogen
- Coombs test
- Positive Coombs test = autoimmune process
- Complete blood count