Thalassaemia intermedia is defined as a group of patients with β thalassaemia whose disease severity varies.
At the severe end of the clinical spectrum of thalassaemia intermedia, patients are usually diagnosed between
the ages of two and six years and, although they survive without regular blood transfusions, growth and
development are impaired. At the other end of the spectrum, there are patients who are completely
asymptomatic until adulthood, when they present with mild anaemia and splenomegaly often found by
chance during haematological examinations or family studies. The diagnosis is dependent on the patient
maintaining a satisfactory haemoglobin (Hb) level at the time of diagnosis without the need for regular blood
transfusions. Patients with severe forms of β thalassaemia intermedia and those patients with thalassaemia
major who had poor access to blood were previously offered splenectomy to help reduce transfusion
requirements. Splenectomy is no longer the mainstay of treatment for these conditions but a considerable
number of both thalassaemia major and intermedia patients have undergone splenectomy