Description

There are several ways of classifying thalassemia, with one of the simplest based on the need to transfuse. Clinically this is helpful since there is such a wide spectrum of mutations and combinations that may be involved. The author is from the University of Oxford.


Main types of NTD thalassemia:

(1) beta-thalassemia intermedia

(2) hemoglobin E beta-thalassemia

(3) hemoglobin H disease

 

Other type of NTD thalassemia:

(4) hemoglobin S beta-thalassemia

(5) hemoglobin C thalassemia

 

Exclusions: hemoglobin S beta-0 thalassemia

 

Clinical features:

(1) limited or no requirements for regular blood transfusion

(2) need for transfusion under certain circumstances (pregnancy, infection, surgery)

(3) normal growth even in the face of low hemoglobin concentrations

 

A child should not be designated transfusion-dependent if there is a need to transfuse during an infection or procedure. The assessment should be performed when the child has recovered and returned to baseline.

 

A patient with NTD thalassemia may have complications (splenomegaly, iron overload, etc) later in life and may show an increased need for blood transfusions.


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