Spinal epidural extramedullary haematopoiesis in β-thalassaemia intermedia

Abstract

SUMMARYA 22-year-old man known to have β-thalassaemia intermedia since childhood presented with bilateral lower limb weakness after spinal anaesthesia for an elective minor operation of his left leg. MRI and CT scans were performed to rule out acute epidural haematoma; coincidental imaging features of marrow hyperplasia and spinal epidural extramedullary haematopoiesis were found. This article will present and discuss the imaging features, differential diagnosis, management and literature review of the rare occurrence of extramedullary haematopoiesis in the spinal epidural space. BACKGROUND

Unraveling a Rare Case of Epidural Extramedullary Hematopoiesis in a Patient With Transfusion-Dependent Beta Thalassemia Presenting With Spinal Cord Compression

“…(Figure 4 ) [ 3 ]. This phenomenon has been documented in chronic hemolytic anemias, myeloproliferative disorders, myelophthisic diseases, hepatocellular cancer, and disorders causing bone marrow disruption [ 4 , 5 ]. Its highest prevalence is observed in beta-thalassemia patients, notably thalassemia intermedia at 20%, with a male-to-female predominance of 5:1 and a usual onset during the fourth decade of life.…”

“…Its highest prevalence is observed in beta-thalassemia patients, notably thalassemia intermedia at 20%, with a male-to-female predominance of 5:1 and a usual onset during the fourth decade of life. It rarely occurs in transfusion-dependent thalassemia major, with incidence rates at 1% [ 4 ]. This discrepancy in prevalence between transfusion-dependent and non-transfusion-dependent thalassemia is probably attributed to the transfusions suppressing the need of EMH to compensate for anemia [ 2 ].…”

Radiological Features of Extramedullary Hematopoiesis in a Young Male With Beta-Thalassemia: A Case Report

“…Extramedullary hematopoiesis rarely occurs in transfusion-dependent thalassemia major, with an incidence rate of only 1% [7]. This discrepancy in prevalence between non-transfusion-dependent and transfusiondependent thalassemia is likely due to transfusions suppressing the need for EMH to compensate for anemia in the latter [3].…”