Spinal Manifestations of Extramedullary Hematopoiesis in Thalassemia

Abstract

BACKGROUND: Thalassemia, a prevalent hemoglobinopathy, is caused by gene mutations resulting in low or dysfunctional alpha (α) and beta (β) globin proteins, leading to extramedullary hematopoiesis (EMH), affecting intra-abdominal organs like the liver and spleen. The EMH can cause spinal canal stenosis, and treatment depends on the patient’s clinical condition, recurrence, and symptom intensity.CASE PRESENTATION: A 27-year-old male patient with back pain, asthenia, and thalassemia had been undergoing medical treatments and repeated blood transfusion since the age of one year. He had undergone splenectomy, tonsillectomy, and adenectomy. The patient was fully conscious, experiencing grade 2 lower limb weakness and sensory level. His abdominal examination was lax and no organomegaly was detected. His chest was clear, and his cardiovascular system (CVS) and electrocardiogram (EKG) showed tachycardia with heart rate (HR)=90/minute. Spinal magnetic resonance imaging (MRI) was done which showed spinal compression from thoracic vertebrae 4 (TV4) to TV10. The patient was operated on for decompressive laminectomy after several blood transfusions. Afterwards, the patient was referred to hematologists.CONCLUSION: Thalassemia results in extramedullary hematopoiesis which might rarely manifest by spinal cord compression. Treatment depends on the patient’s symptoms and the severity of the condition. Decompressive laminectomy is the essential treatment in selected cases.