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Normalisation of total body iron load with very intensive combined chelation reverses cardiac and endocrine complications of thalassaemia major
Author(s) -
Farmaki Kallistheni,
Tzoumari Ioanna,
Pappa Christina,
Chouliaras Giorgos,
Berdoukas Vasilios
Publication year - 2010
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2009.07970.x
Subject(s) - deferiprone , chelation therapy , medicine , endocrine system , ferritin , deferoxamine , gastroenterology , thalassemia , endocrinology , cardiac function curve , cardiology , heart failure , hormone
Summary Cardiac and endocrine disorders are common sequelae of iron overload in transfused thalassaemia patients. Combined chelation with desferrioxamine (DFO) and deferiprone (DFP) is well tolerated and produces an additive/synergistic effect superior to either drug alone. 52 thalassaemia major patients were transitioned from DFO to combined chelation with DFO and DFP. Serum ferritin, cardiac and hepatic iron levels were monitored regularly for up to 7 years, as were cardiac and endocrine function. Patients’ iron load normalized, as judged by ferritin and cardiac and hepatic magnetic resonance imaging findings. In all 12 patients receiving treatment for cardiac dysfunction, symptoms reversed following combined chelation, enabling nine patients to discontinue heart medications. In the 39 patients with abnormal glucose metabolism, 44% normalized. In 18 requiring thyroxine supplementation for hypothyroidism, 10 were able to discontinue, and four reduced their thyroxine dose. In 14 hypogonadal males on testosterone therapy, seven stopped treatment. Of the 19 females, who were hypogonadal on DFO monotherapy, six were able to conceive. Moreover, no patients developed de novo cardiac or endocrine complications. These results suggest that intensive combined chelation normalized patients’ iron load and thereby prevented and reversed cardiac and multiple endocrine complications associated with transfusion iron overload.