Request for therapeutic phlebotomy for secondary hemochromatosis in alpha-thalassemia/HbH disease

The Medical Director of a Transfusion Service received a request to start a therapeutic phlebotomy regimen for a patient in the fifth decade of life with alpha-thal/HbH and hemochromatosis. The patient is not transfusion-dependent, has a mild anemia (Hgb 11), and ferritin about 1000 ng/ml.

The transfusion service physician wonders if phlebotomy even makes sense, since it would stimulate erythropoiesis which would result in accelerated hemolysis and contribute to hemochromatosis. The supposition is that the hemolysis is on-going and of low intensity, and removing the red cells before hemolysis could reduce the iron overload.

A PubMed search found one case report discussing phlebotomy to treat beta-thal with hemochromatosis (De Gobbi. Haematologica 2000;85:865-7). All other articles found discussing the treatment of secondary hemochromatosis in thalassemia recommend chelation therapy, not phlebotomy.

Does anyone have thoughts/experience in phlebotomy to treat secondary hemochromatosis in thalassemia or other congenital hemolytic anemia?

From the Editors: We agree with the poster that there is very little published on this topic and what little is published suggests that these situations often arise out of a misguided attempt to "correct" the thalassemia by giving iron. We also agree that chelation seems more appropriate than phlebotomy given that the defect is enhanced iron absorption.

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