What is thalassemia face?
Changes in facial and cranial bones have been identified as the overexpansion of the bone marrow results in a typical facial appearance. The craniofacial features of thalassemia major patients include larger cheekbones, a rodent or “squirrel-like” face, a depressed nasal bridge, and a protruding maxilla.
Why does chipmunk face occur in patients with thalassemia?
Thalassemia can result in maxillary enlargement, leading to an appearance known as chipmunk face, along with increased spaces between teeth, overbite, and malocclusion. Painful swelling of salivary glands and a dry mouth may occur, which leads to reduced salivary protection and an increased rate of tooth decay.
What is Thalassaemia major?
Thalassemia major is the most severe form of beta thalassemia. It develops when beta globin genes are missing. The symptoms of thalassemia major generally appear before a child’s second birthday. The severe anemia related to this condition can be life-threatening.
What are the two major types of thalassemia?
There are 2 main types of thalassemia: alpha and beta. Different genes are affected for each type. Thalassemia can cause mild or severe anemia. Anemia occurs when your body does not have enough red blood cells or hemoglobin.
What is the difference between thalassemia minor and major?
One mutated gene, you’ll have mild signs and symptoms. This condition is called thalassemia minor or beta-thalassemia. Two mutated genes, your signs and symptoms will be moderate to severe. This condition is called thalassemia major, or Cooley anemia.
Is ESR raised in thalassemia?
Patients with beta thalassemia trait also have a raised ESR. Anemia in TA can be normocytic and normochromic, but our patient presented with microcytic hypochromic anemia which is commonly due to an iron deficiency in females of reproductive age in this part of the world [7].
What is thalassemia major and minor?
If you inherit: One mutated gene, you’ll have mild signs and symptoms. This condition is called thalassemia minor or beta-thalassemia. Two mutated genes, your signs and symptoms will be moderate to severe. This condition is called thalassemia major, or Cooley anemia.
Can two thalassemia minor get married?
Madan said that if two Thalassemia minor patients marry, there were 25 per cent chances that their offspring suffered from Thalassemia major disease and this means that they cannot lead normal life with their life span being cut short to just 25 to 30 years under conditions of regular blood transfusion.
Is alpha thalassemia major or minor?
Low alpha is called alpha thalassemia. Low beta is called beta thalassemia. When the words “trait,” “minor,” “intermedia,” or “major” are used, these words describe how severe the thalassemia is.
Can thalassemia major marry?
Aim: With good care, patients with transfusion-dependent thalassemia major (TDTM) can reach older ages, marry and reproduce.
What is the clinical picture of beta thalassemia major?
Beta thalassemia major is also known as Cooley’s anemia. Today, the classic clinical picture of beta thalassemia major is primarily seen in countries with insufficient resources to provide affected individuals with treatment (e.g. regular transfusions and iron-lowering medications).
What happens to your body when you have thalassemia?
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.
What causes an enlarged spleen in beta thalassemia?
Splenomegaly can cause low levels of the blood cells (platelets) that allow the blood to clot. An enlarged spleen in individuals with beta thalassemia may occur due to increased destruction of red blood cells, the formation of blood cells outside of the bone marrow (extramedullary hematopoiesis), repeated blood transfusions, or iron overload.
How are blood transfusions related to thalassemia major?
Multiple blood transfusions can result in iron overload. The iron overload related to thalassemia may be treated by chelation therapy with the medications deferoxamine, deferiprone, or deferasirox. These treatments have resulted in improving life expectancy in those with thalassemia major.