All published guidelines and standards, by TIF and other national organisations/authorities,
have so far addressed the management of thalassaemia as one clinical entity, and they describe
mainly the management of homozygous beta thalassaemia major and its complications.
It was known for many years that thalassaemia, even homozygous beta thalassaemia, has a
wide spectrum of severity, but there was no clear differentiation in the diagnosis and particularly
management protocols according to severity. Thalassaemia intermedia thus, was for
many decades regarded as just a ‘milder’ form of the disease needing fewer or no transfusion
and consequently less or no iron chelation. With time and extensive research, clinical studies
and observations, it has become clear that these forms, defined as mild on account of their
infrequent or no requirement of regular lifelong blood transfusions during early life, in fact a
great majority of the patients with these forms develop serious complications over time and
require careful monitoring and management which is not necessarily the same as the clinical
care of thalassaemia major. Today, we can safely state that non-transfusion dependent thalassaemia
requires separate attention, early identification and clinical care. TIF has been aware
and has closely followed these developments and in continuation of its policy regarding patient
care, has, requested the authors of this book who are the main pioneers in the field, to produce
separate guidelines for these clinical conditions so that clinicians across the world are made
aware and sensitised to their monitoring and management requirements. Conditions which
can lead to very serious medical complications that affect their survival and quality of life of
patients who are in fact numerically more than those with the major phenotype.