How does Sickle Cell disease affect the Spleen?

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What is Sickle Cell disease?

Sickle cell disease is a condition in which the red blood cells are inherently produced with morphologic deformities. In sickle cell disease, the red blood cells are in the shape of a crescent instead of disk shape. The change in the shape of these cells reduces the surface area and this leads to reduced oxygen transportation. Along with this, the sickled red blood cells also undergo a process called sickling. They form clumps or adhere to the walls of blood vessels and cause obstruction to the blood flow. Due to sickling of cells, there is reduced oxygen flow to the cells and this leads to severe pain in a condition termed as sickle cell crisis.

What are the symptoms of Sickle Cell disease?

Sickle cell disease is an inherited condition. The symptoms are often seen since birth. 

Following are some of the symptoms of sickle cell disease:

·     Anaemia- Due to the destruction of blood cells, and reduced surface area of the cells, there is less oxygen being transported to the body. This condition is called anaemia.

·    Sickle cell crisis: As mentioned above, sickling of cells leads to obstruction of blood vessels which leads to painful episodes. This condition is called Sickle cell crisis. It is most commonly experienced in the limbs and digits and also the chest region.

·   Lowered immunity: The risk of contracting infection is high in patients with sickle cell disease. This is because, the spleen, which is an organ responsible for the immunity of the body is damaged by the sickle cells.

   

   Growth problems: Since the body does not receive enough oxygen, there is deficiency in the growth of the cells. There may even be problems with the development of the individual, both physically and mentally.

·    Problems with vision: The sickling of cells takes place even in the vessels of the retina, leading to vision problems.

How does Sickle Cell disease affect the spleen?

The spleen is an organ which is responsible for the protection of the body and the immune functions.it is located at the upper left side of abdomen and plays a major role in the body’s defence mechanism. In sickle cell disease, the spleen does not function after four to five months of age. This is because the spleen undergoes a phenomenon called splenic sequestration.

What is splenic sequestration?

When sickle cells pass through the blood vessels of the spleen and cause obstruction to the vessels, it is called splenic sequestration. Consequentially, the blood stays within the spleen and the spleen enlarges. The blood count reduces and the patient becomes severely deficient of blood cells. The spleen also enlarges and becomes painful. In most cases of splenic sequestration, the patient’s spleen can be felt.  This condition is also called splenic crisis.

Splenic crisis is seen in patients who have sickle cell disease, right from the age of four months. Splenic sequestration can also occur in patients with thalassemia.

What are the symptoms of splenic sequestration?

The most commonly observed symptoms are:

• ·         Painful and tender spleen
• ·         Enlarged spleen
• ·         Fatigue and weakness
• ·         Irritability
• ·         Paleness due to anaemia
• ·         Reduced immunity
• ·         Not as active as usual

What is the treatment for splenic sequestration?

In splenic sequestration, the child reaches dangerously low levels of haemoglobin and red blood cell count. The spleen enlarges and is painful on palpation. The immediate treatment for splenic sequestration is blood transfusion so that the child is temporarily out of risk. If the enlargement is too much and too frequent, it is advisable to surgically remove the spleen.

Also Read: Can a sickle cell crisis occur in patient with sickle cell trait?

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