Compression secondary to epidural extramedullary haemotopoiesis in thalassemia: a clinical case and review of the literature
Introduction. Extramedullary hematopoiesis generally occurs in a variety of hematological disorders where the normal functioning of the blood forming organs is disturbed. It is a common manifestation in thalassemia where it occurs as a compensatory phenomenon in order to combat long standing anemia. Spinal cord compression as a consequence of extramedullary hematopoiesis in the intraspinal epidural space is an extremely rare complication, though this complication has been reported more commonly in thalassemia. Clinical case. A case of spinal cord compression due to extramedullary hematopoiesis in a patient with thalassemia is reported. The patient was successfully treated with radiation therapy and blood transfusions and he made a complete clinical recovery. Development. The literature is reviewed and the efficacy of several treatments such as surgery, radiotherapy and blood transfusion therapy is discussed. Until recently surgical decompression followed by radiation therapy remained the recommended treatment. Hematopoietic tissue is particularly sensitive to the ionizing radiation and lowdose radiotherapy is enough to relieve the spinal cord compression. Blood transfusion therapy may be diagnostically and therapeutically useful in the management of this entity. Conclusions. A prompt recognition of the syndrome and early treatment with radiotherapy and blood transfusion therapy is recommended to prevent irreversible damage to the spinal cord. Surgery may be only considered in the event of progressive neurological deficit despite of radiotherapy or blood transfusion therapy
Caso clínico Se describe un caso de compresión medular debida a hematopoyesis extramedular en un paciente afecto de talasemia. El paciente fue tratado satisfactoriamente con radioterapia y transfusiones sanguíneas lográndose una total recuperación clínica.
Desarrollo Se revisa la literatura y se discute la eficacia de los distintos tratamientos empleados como la cirugía, radioterapia o las transfusiones sanguíneas. Hasta hace poco tiempo la descompresión quirúrgica seguida por radioterapia era el tratamiento recomendado. El tejido hematopoyético es particularmente sensible a las radiaciones ionizantes y la radioterapia en bajas dosis es suficiente para aliviar la compresión medular producida por él. Las transfusiones sanguíneas pueden ser diagnóstica y terapéuticamente útiles en el manejo de esta entidad.
Conclusiones Se recomienda un diagnóstico precoz de esta entidad y un rápido tratamiento con radioterapia y transfusiones sanguíneas como forma de evitar una lesión medular irreversible. La cirugía sólo se empleará cuando exista un empeoramiento neurológico progresivo a pesar de la radioterapia o de las transfusiones sanguíneas
