Haemolytic anaemia

Saturday, March 25, 2023 4:53 PM

Summary:

• Haemolytic anaemia is characterized by the breakdown of the red blood cells. (haemolysis).

• The haemolysis can either caused by abnormalities in the RBC's (haemoglobin, RBC membrane, intracellular enzymes) which is
called intrinsic haemolytic anaemia. Or by external causes (immune mediated or mechanical damage), which is called extrinsic
haemolytic anaemia.

• Haemolysis can either be categorized depending in where it occur, it may occur inside the blood vessels (intravascular
haemolysis), or in the reticuloendothelial system (extravascular haemolysis), or both.

• Haemolytic anaemia causes varying degrees of fatigue, pallor, weakness, ranging from asymptomatic disease to life threating
haemolytic crisis. Some haemolytic anaemia have more specific finding (thrombosis in paroxysmal nocturnal haemoglobinuria.)

• Haemolytic anaemia should be suspected in anaemia and laboratory finding of haemolysis: (elevated indirect bilirubin and
lactate dehydrogenase reticulocytosis and decrease haptoglobin levels.)

• The coombs test helps to distinguish between immune mediated anaemia (positive) and non immune mediated (negative).

• Treatment involves blood transfusion as required. Additional treatment is based on the type of haemolytic anaemia and it's
causes.

Types and Etiology of haemolytic anaemia:

By RBC pathology
Type Definition Causes
Intrinsic haemolytic Increased destruction of RBC's due to a • RBC membrane defects:
anaemia defect in the RBC's - Hereditary spherocytosis.
- Paroxysmal nocturnal haemoglobinuria.

• Enzyme defects:
- Glucose 6 phosphate dehydrogenase deficiency
(G6PD)
- Pyruvate kinase deficiency (PKD)

• Haemoglobinopathies:
- Sickle cell anaemia.
- Thalassemia.
- Haemoglobin C disease.
- Haemoglobin Zurich.
Extrinsic haemolytic Abnormal breakdown of RBC's • Mechanical destruction in large vessels. (prosthetic
anaemia heart valves).

• Mechanical destruction in small vessels "MAHA"

(HUS, TTP, DIC, HELLP syndrome"

• Autoimmune reactions. (Auto-immune haemolytic

anaemia "AIHA")

• Alloimmune reactions. (Rh incompatibility)

• Immune reactions due to infections (Mycoplasma) or

tumour (chronic lymphocytic leukaemia). (R
 • Infections causing increased destruction of RBC's.
(Babesia, Malaria, Bartonella Bacilliformis.).

• Increased degradation by the spleen. (hypersplenism)

Notes :
• Haemoglobinopathies: a group of dissorder that is characterized by haemoglobin that has an abnormal structure.

• Prosthetic heart valves: a device implanted to replace the patient's native heart valve.

• HELLP syndrome: a complication of pregnancy, and form of preeclampsia that most commonly develop in the third trimester,
and it's characterized by hemolysis, elevated liver enzyme, and low platelets.

• HUS: Hemolytic uric syndrome: it's a condition where the microthrombi occlude in the arterioles and the capillaries, which
results in microangiopathic anemia, thrombocytopenia, and acute kidney injury.

• DIC: Disseminated intravascular coagulation: A condition characterized by systemic activation of the clotting cascade,
platelet consumption, and subsequent exhaustion of clotting factors that leads to widespread thrombosis and hemorrhage.

• Rh incompatibility: A hemolytic disease in which Rh antibodies from Rh-negative mothers transfer to an Rh-positive fetus or
newborn and result in hemolysis.

• Mycoplasma: A type of bacteria characterized by the lack of a cell wall, making them resistant to antibiotics that target cell-
wall synthesis.

• Babesia: A genus of protozoal parasites that infect red blood cells and cause babesiosis "life threating infection". Typically
transmitted via ticks.

• Hypersplenism: A condition characterized by one or more cytopenia caused by the pooling, sequestration, and premature
destruction of blood cells in an enlarged spleen.

By the location of RBC breakdown:

Type Definition Causes
Intravascular hemolytic Increased destruction of the RBC's within • Toxins (snake bites) and oxidizing agents (copper
anemia the blood vessels poisonings).
• G6PD deficiency.

• Antibody mediated hemolysis (transfusion ABO

incompatibility, cold agglutinin disease, hemolytic
anemia of new born)

• Complement mediated hemolysis (PNH).

• Macroangiopathic anemia "mechanical destruction"

(prosthetic cardiac valves)

• Microangiopathic anemia (TTP, HUS, HELLP syndrome,

systemic lupus erythrematus)
Extravascular hemolytic Increased destruction of the RBC's within • Antibody mediated hemolysis (worm and cold
anemia the reticuloendothelial system. (primarily in agglutinin disease.).
the spleen)
• RBC defects (sickle cell anemia, spherocytosis, PKD)

Clinical features:
Signs of anemia:
• Pallor. (paleness of the skin and/ or mucous membrane)

• Fatigue.

Signs of hemolysis:
• Jaundice. (a yellow discoloration of the skin, sclerae, and mucous membrane due to the deposition of the bilirubin)

• Pigmented gallstones.(A type of gallstone composed primarily of calcium bilirubinate (unconjugated bilirubin and calcium))

• Splenomegaly. (an enlargement of the spleen)

• Hemoglobinuria. (the presence of hemoglobin in the urea, a common cause is intravascular hemolytic anemia)

Diagnosis:

‫ ﻻ ﺛﻧ‬Ê Ê ‫ﻷ ﺣ‬