Does thalassemia intermedia need blood transfusion?

Does thalassemia intermedia need blood transfusion?

Although individuals with thalassaemia intermedia do not usually need regular blood transfusions, there is some evidence that complications, particularly later in life, may be less common in regularly transfused patients.

What is non transfusion dependent thalassemia?

Non-transfusion-dependent thalassemias (NTDT) is a term used to label patients who do not require lifelong regular transfusions for survival, although they may require occasional or even frequent transfusions in certain clinical settings and usually for defined periods of time (Figure 1).

How can you distinguish between thalassemia major and intermedia?

Individuals with beta thalassemia major require regular blood transfusions, as frequently as every 2-4 weeks in severe cases. Individuals with beta thalassemia intermedia occasionally require blood transfusions such as when suffering from an illness or infection or when planning to undergo surgery.

Can thalassemia minor become Intermedia?

Thalassemia can be broken into three categories: Trait: Person carries the gene for mutation but does not have the disease. This is sometimes called thalassemia minor. Intermedia: Person with thalassemia somewhere between trait and major.

How do you treat thalassemia intermedia?

Splenectomy is usually needed at some time because of hypersplenism and mechanical encumbrance. Reactivation of HbF is possible only in a small proportion of patients: hydroxycarbamide (also known as hydroxyurea) appears to be the most effective drug for this purpose.

What is beta thalassemia intermedia?

β-Thalassemia intermedia is a clinical condition of intermediate gravity between β-thalassemia minor, the asymptomatic carrier, and β-thalassemia major, the transfusion-dependent severe anemia. It is characterized by a significant clinical polymorphism, which is attributable to its genetic heterogeneity.

What is transfusion dependent thalassemia?

Transfusion-dependent β-thalassemia (TDT) is the most severe form of the disease, characterized by severe anemia, and requiresiii lifelong, regular blood transfusions to maintain Hb levels. These transfusions carry the risk of progressive multi-organ damage due to unavoidable iron overload.

How is thalassemia intermedia treated?

Blood transfusion and chelation are necessary in some patients, especially during childhood, in order to promote growth and prevent bone deformities. Alloimunisation, however, is frequent and can be difficult to control. Splenectomy is usually needed at some time because of hypersplenism and mechanical encumbrance.

How can you tell the difference between major and minor thalassemia?

If you inherit:

1. One mutated gene, you’ll have mild signs and symptoms. This condition is called thalassemia minor or beta-thalassemia.
2. Two mutated genes, your signs and symptoms will be moderate to severe. This condition is called thalassemia major, or Cooley anemia.

Does thalassemia minor affect pregnancy?

During pregnancy, women with thalassemia minor will often show more significant anemia, which is often most prominent during the latter half of the second trimester and early third trimester (4-7).

Do you need transfusions for non transfusion dependent thalassemia?

Correspondence: [email protected] Non-transfusion-dependent thalassemias include a variety of phenotypes that, unlike patients with beta (β)-tha- lassemia major, do not require regular transfusion therapy for survival.

What is the difference between thalassemia intermedia and major?

Key Takeaways Thalassemia intermedia is a milder form of thalassemia, wherein there is a reduced production of the “adult” hemoglobin, which leads to anemia. Thalassemia intermedia is less severe than thalassemia major, which needs a long-term transfusion of red blood cells.

How often does ntdt form of α thalassaemia occur?

The annual number of births for the NTDT form of α-thalassaemia, α-thalassaemia intermedia or haemoglobin H disease, is approximately 10,000 [8, 11]. However, these numbers might even be underestimated since there are limited genetic epidemiology studies of α-thalassaemia syndromes in several parts of the world in the Far East, in particular.

What is the diagnosis of β-thalassaemia major?

For example, the diagnosis of β-thalassaemia major entails life- long regular transfusion requirement for survival, and the term transfusion-dependent thalas- saemia (TDT) is conventionally used to describe such forms [3].