Why does liver enlarge in thalassemia?

Why does liver enlarge in thalassemia?

Iron overload This may be due to the frequent blood transfusions or the disease itself. Iron overload raises the risk of hepatitis, (swollen liver), fibrosis (scarring in the liver), and cirrhosis, or progressive liver damage due to scarring. The endocrine glands produce hormones.

How does thalassemia affect liver?

Based on current data, patients with thalassemia have several established risk factors for the development of hepatocarcinoma (HCC), which include liver iron overload and high prevalence of viral hepatitis with or without cirrhosis.

Why does thalassemia cause hyperbilirubinemia?

Individuals with thalassemia have hyperbilirubinemia secondary to ongoing hemolysis and ineffective erythropoiesis. Iron loading occurs because of increased iron absorption as well as the administration of blood transfusions.

Does thalassemia minor affect the liver?

People with thalassemia can get too much iron in their bodies (iron overload), either from frequent blood transfusions or from the disease itself. Too much iron can cause damage to your heart, liver, and endocrine system, which includes glands that produce hormones that regulate processes throughout your body.

Is thalassemia related to liver?

Current HBsAg positivity in thalassaemia major ranges from <1% to >20% and Hepatitis B infection remains a significant cause of chronic liver disease and hepatocellular carcinoma in patients with thalassaemia in many regions of the developing world.

Is thalassemia a liver disease?

Abstract. Liver disease is the second cause of death for thalassemia major (TM) patients, mainly due to HCV infection and transfusional iron overload. Few data are so far available for thalassemia intermedia (TI) patients who are much less transfused but, because of chronic anemia they have an increased iron absorption …

What organs does thalassemia affect?

Thalassemia can cause bone deformities in the face and skull. People who have thalassemia may also have severe osteoporosis (brittle bones). Too much iron in your blood. This can cause damage to the heart, liver, or endocrine system (glands in the body that make hormones, like the thyroid gland and adrenal glands).

What is thalassemia trait?

Thalassemia (thal-uh-SEE-me-uh) is an inherited blood disorder that causes your body to have less hemoglobin than normal. Hemoglobin enables red blood cells to carry oxygen. Thalassemia can cause anemia, leaving you fatigued. If you have mild thalassemia, you might not need treatment.

How does thalassemia affect the production of HB?

Thalassemia results when mutations affecting the genes involved in Hb biosynthesis lead to decreased Hb production. The clinical phenotype results from both the diminished amount of the particular globin chain as well as from the resultant chain imbalance that occurs because of normal production of the other globin chain.

What are the causes of hepatomegaly in liver?

What are the causes of hepatomegaly? Hepatomegaly is often a sign that the tissue within the liver isn’t functioning properly. Taking certain medications, such as amiodarone and statins, may also cause liver injury. metastatic cancer, or cancer that starts in other organs and spreads to the liver

What are the symptoms of beta thalassemia major?

Beta thalassemia major is caused by a homozygous mutation (beta-zero thalassemia) of the beta-globin gene, resulting in the total absence of beta chains. It manifests clinically as jaundice, growth retardation, hepatosplenomegaly, endocrine abnormalities, and severe anemia requiring life-long blood transfusions.

What is the genetic cause of alpha thalassemia?

It is caused by either a genetic mutation or a deletion of certain key gene fragments. Alpha thalassemiais caused by alpha-globin gene deletion which results in reduced or absent production of alpha-globin chains. Alpha globin gene has 4 alleles and disease severity ranges from mild to severe depending on the number of deletions of the alleles.

How are hemoglobin molecules affected by thalassemia?

Hemoglobin molecules are made of chains called alpha and beta chains that can be affected by mutations. In thalassemia, the production of either the alpha or beta chains are reduced, resulting in either alpha-thalassemia or beta-thalassemia.

What are the causes of hepatomegaly? Hepatomegaly is often a sign that the tissue within the liver isn’t functioning properly. Taking certain medications, such as amiodarone and statins, may also cause liver injury. metastatic cancer, or cancer that starts in other organs and spreads to the liver

What causes the spleen to be enlarged in thalassemia?

Complications. Enlarged spleen (splenomegaly). The spleen helps your body fight infection and filter unwanted material, such as old or damaged blood cells. Thalassemia is often accompanied by the destruction of a large number of red blood cells. This causes your spleen to enlarge and work harder than normal.

What is the phenotype of beta thalassemia intermedia?

Beta thalassemia intermedia: An in between clinical phenotype with heterogenous genetic mutations that still allow for some Beta chain production (e.g. B+/B0, B+/B+). Some rare cases also exist in which both beta and alpha mutations coexist