Spinal cord compression due to extramedullary haematopoiesis in two patients with thalassaemia: complete regression with blood transfusion therapy

Abstract

Extramedullary haematopoiesis, a common finding in thalassaemia, is rarely localized in the spinal cord and even more rarely has neurological manifestations. We present two patients suffering from thalassaemia (intermedia and beta homozygous) and paraparesis due to spinal cord compression from intrathoracic extramedullary haematopoietic masses. Diagnosis was based on magnetic resonance imaging findings, and treatment consisted of blood hypertransfusions. The majority of published cases have been successfully treated by radiation and in some cases by blood transfusions. Our two patients completely recovered, and there has been no recurrence during the 4 years since their treatment. Diagnosis and treatment of this rare condition are discussed.

Beta-Thalassemia Presenting as an Acute Neurological Complication: A Case Report

“…However, the resolution was too rapid to be attributable to steroids alone. It has been shown that hyper-transfusion therapy helps to shrink extra-medullary hematopoietic tumors and alleviate the compressive effect on spinal cord [3,5,6,9].…”

“…The resultant anemia, hemolysis and ineffective erythropoiesis form the fundamental pathology for clinical manifestations of beta-thalassemia. In extreme cases of dyserythropoiesis, extra-medullary hematopoietic tumors may develop [3][4][5][6]. Acute neurological complications in patients with beta-thalassemia have been reported in association with cerebral ischemia, spinal cord fractures and compression from extra-medullary hematopoietic tumors [7][8][9][10][11].…”

A 26‐yr-old female with persistent cough

“…The management of spinal cord compression remains controversial. Radiotherapy with blood transfusions [12], hypertransfusion with hydroxyurea therapy [13,14], or only blood transfusions [11,15] were found to be successful in the complete regression of haematopoietic masses. The different treatment strategies for symptomatic pleural effusion and haemothorax at intrathoracic EMH include pleurocentesis [9], tube thoracostomy [1], pleurodesis with tetracycline [16], and low-dose radiation [17].…”

“…Noninvasive studies, including chest roentgenograms, CT and magnetic resonance imaging, can establish the EMH in most cases [10,11,18]. Bone marrow scanning with TC-99M sulfur colloid is a convenient noninvasive method of further establishing the diagnosis [2,8,19].…”

“…Massive haemothorax [1,5], symptomatic pleural effusion [2,9], and spinal cord compression [11] are complications of intrathoracic EMH.…”