Literature DB >> 16462720

Paraplegia due to spinal cord compression by extramedullary erythropoietic tissue in a thalassaemia intermedia patient with gynecomastia secondary to cirrhosis: successful treatment with hydroxyurea.

Maria R Gamberini1, Monica Fortini, Vincenzo De Sanctis.   

Abstract

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.

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Year:  2004        PMID: 16462720

Source DB:  PubMed          Journal:  Pediatr Endocrinol Rev        ISSN: 1565-4753


  3 in total

Review 1.  β-Thalassemia intermedia: a comprehensive overview and novel approaches.

Authors:  Chingiz Asadov; Zohra Alimirzoeva; Tahira Mammadova; Gunay Aliyeva; Shahla Gafarova; Jeyhun Mammadov
Journal:  Int J Hematol       Date:  2018-01-29       Impact factor: 2.490

2.  Long segment spinal epidural extramedullary hematopoiesis.

Authors:  Kanwaljeet Garg; Pankaj Kumar Singh; Manmohan Singh; P Sarat Chandra; Bhawani Shankar Sharma
Journal:  Surg Neurol Int       Date:  2013-12-26

3.  Spinal cord compression due to extramedullary hematopoiesis in a patient with E-beta-thalassemia managed without radiation or surgery.

Authors:  Van T La; Michael Diatte; Johnathan Gaston; Dallas Dick; Raed Sweiss; Zahra Pakbaz
Journal:  J Community Hosp Intern Med Perspect       Date:  2018-08-23
  3 in total

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