Literature DB >> 8428551

[Reversible spinal cord compression caused by extramedullary hematopoietic foci in thalassemia].

G Massenkeil1, W Wichmann, F Krummenacher, K Rhyner.   

Abstract

A now 42-year-old Thai woman was known to have been anaemic since childhood. When aged 33 years she was diagnosed as having beta zero/HBE thalassaemia. Computed tomography demonstrated a tumour in the posterior mediastinum, histologically found to be an extramedullary haematopoietic focus. Subcutaneous infusion of deferoxamine (2 g five times weekly), initiated because of massive iron overload, reduced the serum ferritin level from 3,460 ng/ml to less than 500 ng/ml. The haemoglobin level in the subsequent years was between 6 and 8 g/dl. Five years later sensory deficits were noted from the 5th thoracic vertebra downwards. Magnetic resonance imaging demonstrated a tumour which compressed the spinal cord: it, too, was an ectopic haematopoietic focus. The neurological symptoms disappeared after radiotherapy with 3,000 cGy, but they recurred 4 years later. Because of the low radiation reserve of the spinal cord, hypertransfusion treatment was initiated, namely 16 RBC concentrates within 4 months and afterwards two transfusions every 3 months. By this means the haemoglobin level was kept at about 9 g/dl. The tumour had regressed 4 months after onset of treatment. For 2 years since the beginning of the hypertransfusion treatment the patient has remained free of neurological symptoms.

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Year:  1993        PMID: 8428551     DOI: 10.1055/s-2008-1059306

Source DB:  PubMed          Journal:  Dtsch Med Wochenschr        ISSN: 0012-0472            Impact factor:   0.628


  2 in total

1.  Spinal cord compression due to extramedullary haematopoiesis in thalassaemia: a case report and review of the literature.

Authors:  I S Martina; P A van Doorn
Journal:  J Neurol       Date:  1996-04       Impact factor: 4.849

2.  Watershed Infarct in Beta-Thalassemia Major Patient.

Authors:  Behnaz Ansari; Mohammad Saadatnia; Ali Asghar Okhovat
Journal:  Case Rep Neurol Med       Date:  2017-03-27
  2 in total

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