Proteinuria in haemoglobin H disease

الملخص EN

While extensive studies are available on the renal manifestations in sickle cell disease, there is no documented evidence in favour of renal changes in the commonly encountered haemoglobinopathy namely, α thalassaemia.

A light, immuno-flourescence and electron microscopic observations on a renal biopsy of a young male patient who is a known case of Hemoglobin H disease with proteinuria is documented.

This is probably the first case reported, primarily for renal complications with demonstrable podocyte abnormalities.

The pathogenesis of renal manifestations remains obscure.

The renal alterations are less likely to be due to minimal change disease in view of immunoglobulin deposits in the mesangial area.

However involvement of any other immune process or continued sub- clinical haemolysis producing proteinuria remain to be seen on long-term studies.