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

Dtsch Med Wochenschr. 1993 Jan 29;118(4):100-6. doi: 10.1055/s-2008-1059306.
[Article in German]

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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Blood Transfusion
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Erythrocyte Transfusion
  • Female
  • Germany, West
  • Hematopoiesis, Extramedullary*
  • Heterozygote
  • Humans
  • Remission Induction
  • Spinal Cord Compression / diagnosis
  • Spinal Cord Compression / etiology*
  • Spinal Cord Compression / therapy
  • Thailand / ethnology
  • beta-Thalassemia / complications*
  • beta-Thalassemia / diagnosis
  • beta-Thalassemia / therapy