Thalassemia differs from sickle-cell anemia

Thalassemia differs from sickle-cell anemia

 

In both sickle-cell anemia and thalassemia, there are defects and errors in the genes responsible for the production of red blood cell hemoglobin, but thalassemia and sickle-cell anemia have differences that we will explain the differences between the two in this article.

 

Hemoglobin is responsible for delivering oxygen to the cells of our body, so a defect in the structure of hemoglobin disrupts oxygen supply and will lead to fatigue.

In the sickle-cell anemia, even the shape of red blood cells becomes abnormal and causes liver problems and so on.

 

Heredity plays a key role in both thalassemia and the sickle-cell anemia. For example, in thalassemia, both parents are more likely to have a baby with a more severe form of thalassemia. In very severe cases of thalassemia, even the fetus dies in the mother's womb.

 

The differences between thalassemia and sickle-cell anemia are as follows:

 

 

Thalassemia

 

Sickle-cell anemia

 

Thalassemia:

Reasons:

An abnormal mutation in one of the genes responsible for making the protein part of red blood cell hemoglobin.

 

You will get this genetic error from your parents:

  • If your parent carries a defective gene, their child may have thalassemia minor. A person with thalassemia minor has no symptoms but is himself a carrier of the defective gene to later generations. Some people with thalassemia minor may have simple and basic symptoms, such as mild anemia.

  • If both parents are carriers of the defective gene, their children are more likely to develop more severe forms of thalassemia.

 

Regions of the world in terms of the prevalence of thalassemia:

Asian, Middle Eastern, African, and Mediterranean inhabitants, such as Turkey and Greece

 

What are the types of thalassemia?

Beta thalassemia (mutation in the beta chain gene of the protein part of hemoglobin)

  • Beta thalassemia major
  • Beta-thalassemia intermedia

 

Alpha thalassemia (mutation in the gene of the alpha chains of the protein part of hemoglobin)

  • Hemoglobin H
  • Hydrops fetalis (the severity of the defect is so high that the fetus dies in utero)

Thalassemia minor

 

What are the symptoms of thalassemia?

  • Symptoms of beta thalassemia major:

Symptoms begin at birth of the affected baby Severe and life-threatening anemia will be evident from the demonstrations.

Other symptoms of beta thalassemia major include:

  • Pallor
  • fussiness
  • Recurrent infections
  • Delay and inability of the child to walk
  • Low appetite
  • Jaundice

 

Thalassemia differs from sickle-cell anemia

 

  • Enlarged liver

 

These people will need regular blood transfusions, Deferoxamine prevents the effects of iron accumulation in these people, Deferoxamine is very vital.

 

  • Beta-thalassemia intermedia

The symptoms will be mild and people with the condition will not need a blood transfusion.

 

  • Alpha Thalassemia

 

  • Hemoglobin H:

Changes in the bones due to increased bone marrow hematopoietic activity, to compensate for anemia, cause the growth of the cheeks, forehead and jaw, and the shape of the affected person's face becomes special.

  • Jaundice
  • extremely enlarged spleen
  • malnutrition

 

  • Hydrops fetalis:

Hydrops fetalis is a very severe form of genetic disorder in the production of hemoglobin protein, which in most cases leads to the death of the infected fetus in the mother's womb and Sometimes an infected baby dies shortly after birth.

 

Thalassemias cause anemia by disrupting the structure of red blood cells hemoglobin; symptoms of anemia include:

  • Lightheadedness
  • Fatigue
  • Weakness
  • Shortness of breath

 

Children with thalassemia:

  • They are pale
  • They don’t have appetite
  • They have poor growth
  • They have jaundice

 

Hemoglobin is a part inside the structure of red blood cells and consists of two parts:

  1. the heme :Iron
  2. the globin: Protein section

Globin is made up of beta and alpha chains, and the gene for beta is different from the gene for alpha.

Disorders and mutations in beta-producing genes lead to beta thalassemia, and disorders and mutations in alpha-producing genes lead to alpha thalassemia.

 

Sickle-cell anemia:

Sickle-cell anemia or SCD (sickle-cell disease) is a genetic disorder that causes RBCs to change shape and become a nuisance.

In a healthy person, the RBCs are disc-shaped. These discs also have the flexibility to pass through very narrow vessels. The defective genes in the SCD cause the RBCs to deform and become crescent-shaped.

abnormal crescent shape resembling a sickle

These RBCs can no longer be flexible in crossing narrow paths. RBCs in the SCD become very hard and rigid and get stuck in the small arteries. Clogged arteries are associated with pain attacks.

 

What are the symptoms of SCD?

Symptoms may occur in infants under four months of age, but it is usually around six months when it becomes symptomatic.

Common symptoms between different SCD types include:

  • Excessive fatigue
  • Irritability
  • fussiness
  • Swelling of the hands and feet
  • Chest pain
  • Pain in the legs
  • Pain in the arms

 

Thalassemia differs from sickle-cell anemia

 

  • Recurrent infections
  • bed wetting

 

Populations in which SCD is more common:

  • Africans
  • Indians
  • Originally from Saudi Arabia
  • Inhabitants of countries around the Mediterranean

 

Types of SCD

There are several types of SCDs that are not covered here:

SS, SC, SD, SE, SO and SB+, SB0

And sickle cell trait Complications of SCD occur when the RBCs, with their abnormal, rigid and inflexible shape, get stuck inside the blood vessels.

These attacks are called sickle-cell crisis which are very painful and sometimes cause serious damage to organs that have blocked their arteries.

 

SCD complications:

Severe anemia:

In sickle-cell anemia, red blood cells break down quickly and die, Normal RBCs have a lifespan of one hundred and twenty days, but in a sickle-cell disorder, RBCs die early and last only ten to twenty days.

 

Hand foot syndrome:

When defective RBCs get stuck inside the small veins in the legs and arms, the hands and feet become swollen, Persistence of blood is called blood stasis, which can cause sores on the hands and feet.

Sometimes this is the first manifestation of sickle-cell.

 

Splenic sequestration

Trapped defective RBCs inside the splenic arteries cause vascular occlusion, which results in excessive enlargement of the spleen and pain.

 

Growth retardation:

And puberty is delayed.

 

Neurological problems:

  • Convulsions
  • Stroke
  • coma

It can be a consequence of cerebrovascular occlusion.

Obstruction of the arteries of the eye can lead to blindness.

 

Cardiovascular problems

  • Arrhythmias
  • Heart attack and heart failure
  • Pulmonary hypertension and respiratory problems
  • Skin lesions
  • Gallstones

Priapism (In men, trapping defective RBCs in the arteries of the penis can lead to a long, painful erection called priapism).

 

Influencing factors in attacks and sickle-cell crisis

The following factors may trigger attacks and sickle-cell crisis:

  • Stresses
  • Illnesses
  • Dehydration of the body
  • Ambient temperature changes
  • Climb to heights

And the important point is that people with sickle-cell disorder may suffer:

 

Sickle chest syndrome

The most severe complication of the sickle-cell crisis.

It is accompanied by severe chest pain, fever, cough, shortness of breath and excessive sputum.

People who experience this sign have a worse prognosis than other people with sickle-cell disorder, which means they are more likely to have more complications in the future.

 

Diagnosis of cell cycle disorder:

Clinical manifestations and symptoms along with a blood test that shows anemia.

Low hemoglobin 6 8gr / dL

Examination of the blood smear under a microscope, which shows abnormal and sickle-shaped shapes.

Other specific tests, such as the Sickle Solubility Test, show the presence of defective hemoglobin.

Hemoglobin electrophoresis and...

 

Treatment of sickle-cell disorder:

This disorder, like other genetic disorders, has no cure.

Treatments are supportive to reduce the severity of symptoms and prevent attacks and complications:

  • Pain Relief Medications

 

Thalassemia differs from sickle-cell anemia

 

  • Complete blood transfusion
  • Auxiliary oxygen: Oxygen Mask

 

Thalassemia differs from sickle-cell anemia

  • Bone marrow transplant

 

Ways to help at home:

  • Do not let to be dehydrated.
  • Take folic acid under a doctor's supervision
  • Be physically active.
  • Eat a diet full of natural and healthy ingredients.
  • Tell your doctor as soon as any infection starts.

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Address: 393 University Avenue,Suite 200,Toronto ON MG5 2M2,CANADA

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Phone: +1(647)303 0740

All Rights Reserved © By MarsoClinic

Terms of Use