[Reversible spinal cord compression caused by extramedullary hematopoietic foci in thalassemia].
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.
Massenkeil
G
G
Departement für Innere Medizin, Universitätsspital Zürich.
Wichmann
W
W
Krummenacher
F
F
Rhyner
K
K
D000328:Adult; D001803:Blood Transfusion; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D017707:Erythrocyte Transfusion; D005260:Female; D005860:Germany, West; D006411:Hematopoiesis, Extramedullary; D006579:Heterozygote; D006801:Humans; D012074:Remission Induction; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D013785:Thailand / Q000208:ethnology; D017086:beta-Thalassemia / Q000150:complications* / Q000175:diagnosis / Q000628:therapy