Hypertransfusion: a successful method of treatment in thalassemia intermedia patients with spinal cord compression secondary to extramedullary hematopoiesis.

Abstract

Extramedullary hematopoiesis is a common compensatory phenomenon to chronic hemolytic anemias including thalassemia. Several sites can be involved including the liver, the spleen, the lymph nodes, and other less common locations. Spinal cord compression may result in the rare cases wherein the hematopoietic develops intraspinally. Treatment of such conditions still is controversial. This article reviews the literature and reports two cases of thalassemia intermedia involving patients who presented with neurologic symptoms after acute spinal cord compression secondary to extramedullary hematopoiesis. The diagnosis was established by magnetic resonance imaging. Hypertransfusion therapy was used as our first-line treatment method. Complete neurologic recovery was achieved. Improvement in the neurologic status started as soon as the first week of treatment. Clinical awareness is important for early diagnosis and prevention of irreversible neurologic complications in such cases. Magnetic resonance imaging is the radiologic method of choice for diagnosing extramedullary hematopoietic masses and for delineating the extent of spinal cord involvement. Hypertransfusion seems to be a promising treatment method that should be recommended as a first-line approach or as an adjuvant therapy to other methods.