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What is haemolytic anaemia?

A number of conditions that result in the premature destruction of RBCs.
Presents with acute or subacute development of fatigue or jaundice, and may include orthostasis and mild splenomegaly.
NB: Usually macrocytic anaemia, with↑reticulocyte count and jaundice with unconjugated hyperbilirubinaemia


What indicated haemolytic anaemia?

Anaemia, reticulocytosis, low haptoglobin, high LDH and high indirect bilirubin suggest haemolysis.


What does Coomb's test do?

Differentiates immune from non-immune aetiologies.


Causes of haemolytic anaemia?

Inherited RBCs defects (membrane)- hereditary spherocytosis, elliptocytosis (pyropoikilocytosis)
Enzyme deficiencies- G6PD deficiency, Pyruvate kinase deficiency.
Abnormal Hb production- sickle cell anaemia, thalassaemia
Immune- Autoimmune (warm or cold reacting antibodies, drug-induced, autoimmune)
Non-immune- infection, trauma (like malignant HTN), HELLP syndrome in pregnancy, mechanical prosthetic heart valves, march haemolysis, thermal injury and osmotic lysis, hypersplenism and liver disease.


Signs & symptoms of haemolytic anaemia?

Shortness of breath
Active infections
Episodic dark urine
Triggered by exposure to cold


Risk factors of haemolytic anaemia

Autoimmune disorders
Lymphoproliferative disorders
Prosthetic heart valve
Family origin in the Mediterranean
Middle East
Sub-Saharan Africa
Southeast Asia
FHx of haemoglobinopathy or RBC membrane defects
Paroxysmal nocturnal haemoglobinuria
Thermal injury


Tests and investigations of haemolytic anaemia

FBC (low Hb)
MCHC (increased)
Reticulocyte count
Peripheral smear
Unconjugated bilirubin
Direct antiglobulin test (DAT or Coombs)


What is the Coombs test?

It tests for antibodies to RBCs.
There's indirect and direct Coombs test.


What is the Indirect Coombs test?

This is the test used to cross match blood for suitability for transfusion.
A positive test means the tested donor red cells are incompatible and should not be given as a transfusion.
Blood sample from donor and recipient’s serum (containing antibodies), are obtained and mixed together.
Antihuman antibodies (Coombs reagent) are added to the mixture.
If RBCs agglutinate, the test is positive.


When is indirect Coombs test used?

Prenatal testing for pregnant women.
Testing blood prior to a transfusion.


What is the direct Coombs test?

This test is done much less often and detects
antibody to patient’s own serum causing an autoimmune haemolytic anaemia.
Blood sample from patient with immune-mediated haemolytic anaemia is washed to
extract RBCs.
RBCs incubated with antihuman antibodies (Coomb’s reagent).
If RBCs agglutinate, the test is positive


When is direct Coombs test used?

Used to identify haemolytic anaemia.


Differentials of haemolytic anaemia?

Anaemia due to blood loss
Underproduction anaemia
Transfusion reaction.


Management of haemolytic anaemia?

Once the diagnosis of haemolytic anaemia has been determined, a haematological
consultation is warranted.

All aetiologies of haemolytic anaemia require some degree of supportive care- supportive care includes transfusions and folate supplementation.


What is hereditary spherocytosis?

autosomal dominant, commonest inherited RBC disorder in northern Europeans.
RBCs are less deformable and get trapped in the spleen, causing extravascular haemolysis and splenomegaly.
Blood film shows micro-spherocytes and polychromatic macrocytes
(reticulocytes), with no areas of central pallor


Diagnosis of hereditary spherocytosis?

EMA-binding test is gold standard but if family history + blood film + FBC results correlate, it is not required.


Complications of hereditary spherocytosis

Neonatal and adult jaundice
Chronic haemolysis
Increased risk of gallstones from increased bilirubin


Management of hereditary spherocytosis?

Regular folic acid supplementation, often splenectomy required.


What is hereditary elliptocytosis?

Hereditary elliptocytosis
(pyropoikilocytosis) –autosomal dominant,
incidence. Not clinically significant except in homozygotes
Also known as ovalocytosis.
In most cases treatment is not necessary.
In the most severe variants, folic acid, red cell transfusion and splenectomy (after the age of 5) may be required. Patients should be monitored during events known to precipitate haemolysis.


Pathophysiology of hereditary elliptocytosis?

a group of disorders of the red blood cell (RBC) membrane that are characterized by elliptical-shaped erythrocytes (elliptocytes) and shortened RBC survival.
Unlike normal RBCs, which repeatedly and momentarily assume an elliptical shape to negotiate through capillaries but then regain their biconcave discoid shape after they pass through the microcirculation, the RBCs in HE lack the elastic recoil necessary for returning to the discoid shape and eventually assume the fixed characteristic morphology of elliptocytes, with a decreased surface-to-volume ratio.
These elliptocytes are not as deformable as normal RBCs and are eventually trapped and removed by the spleen; this manifests as haemolytic anaemia.


What is hereditary stomacytosis?

Hereditary stomatocytosis is characterized by excessively water-laden erythrocytes.
The extremely high influx of Na+(sodium) and water from the plasma exceeds the loss of cellular K+ (potassium ion) and the resulting swollen red cells show a unique combination of large size (high mean corpuscular volume [MCV]), low mean corpuscular hemoglobin concentration (MCHC), and osmotic sensitivity in the osmotic fragility test.
No other condition shows this unique combination. Blood smears feature stomatocytes, or a mixture of stomatocytes and spherocytes.


What is G6PD deficiency?

X-linked recessive disease.
Blood film shows bite-cells
and blister-cells, after removal of Heinz bodies (denatured Hb) from RBCs.
May also show contracted RBCs.


What does G6PD deficiency precipitated by?

Precipitated by:
Drugs–aspirin, primaquine, sulphonamides, nitrofurantoin, vitamin K
Exposure – broad beans/favism, moth balls, henna


Diagnosis of G6PD deficiency

Enzyme assay for G6PD levels >8 weeks after crisis (as young RBCs may have enough enzyme so results normal)


Management of G6PD deficiency?

Avoid precipitants and transfuse blood if severe.


What is pyruvate kinase deficiency?

Autosomal recessive disease.
↓ATP production causes↓RBC survival.
Presents with neonatal jaundice, later chronic haemolysis with splenomegaly ± jaundice.
Blood film shows “Sputnik” cells, with spiky
Diagnosis: Enzyme assay


Treatment of pyruvate kinase deficiency?

Often not needed, but splenectomy may help.


What is autoimmune haemolysis (AIHA)?

Mediated by autoantibodies causing extravascular haemolysis and spherocytosis.


How many types of AIHA?



What is warm AIHA?

IgG-mediated binds to RBCs at body temperature (37 deg).
Tx: Steroids or immunosuppressants, ± splenectomy. Prednisolone 1mg/kg OD mane, weaning off slowly. Give omeprazole 20mg OD for gastroprotection.