Paraplegia due to spinal cord compression by extramedullary erythropoietic tissue in a thalassaemia intermedia patient with gynecomastia secondary to cirrhosis: successful treatment with hydroxyurea.
We report a 24 years old patient with thalassaemia intermedia and gynecomastia, complicated by paraplegia and urinary/fecal incontinence due to spinal cord compression by an extramedullary erythropoiesis (EE) mass, treated with long-term hydroxyurea (HU). Neurological improvement occurred during the first 6 weeks of HU therapy (1,000 mg/day) and magnetic resonance imaging showed a reduction in EE mass. HU dosage was reduced to 500 mg/day after 5 months, and treatment was discontinued after 25 months. Five months later there was a partial recurrence of neurological symptoms, which responded to radiotherapy. HU may have a role in the symptomatic treatment of spinal cord compression due to EE, particularly when radiotherapy is unavailable.
Gamberini
Maria R
MR
Department of Reproduction and Growth, Paediatric and Adolescent Unit, S. Anna Hospital, Ferrara, Italy. mr.gamberini@libero.it
Fortini
Monica
M
De Sanctis
Vincenzo
V
D000328:Adult; D000986:Antisickling Agents / Q000627:therapeutic use; D004920:Erythropoiesis / Q000187:drug effects; D006177:Gynecomastia / Q000150:complications*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000150:complications; D008297:Male; D010264:Paraplegia / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D013117:Spinal Cord Compression / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D017086:beta-Thalassemia / Q000150:complications*
D000986:Antisickling Agents; D006918:Hydroxyurea