EXTRAMEDULLARY HAEMOPOIESIS LEADING TO SPINAL CORD COMPRESSION IN A PEDIATRIC PATIENT WITH BETA THALASSAEMIA MAJOR
Keywords:
Beta thalassemia major, Spinal cord compression, Extramedullary hematopoiesis, Surgical decompressionAbstract
Beta thalassemia major, a severe form of the disease, is characterized by significant clinical manifestations, including ineffective erythropoiesis &chronic anaemia. Extramedullary haematopoiesis (EMH) is a rare but serious complication in patients with chronic anaemias, such as beta thalassemia major. More commonly associated with beta thalassemia major, EMH can lead to significant clinical manifestations. We present the case of a 12-year-old boy with beta thalassemia major on Hydroxyurea who experienced a 3-month history of backache, progressive lower limb weakness, and urinary incontinence. MRI revealed multilevel large soft tissue masses within the spinal canal causing spinal cord compression. The patient underwent T4-T8 posterior decompression surgery, which led to significant clinical improvement. Histopathology confirmed the presence of trilineage haematopoiesis without evidence of neoplastic processes. Follow-up MRI at 8-weeks post-surgery showed a marked reduction in the masses and no significant spinal cord compression. This case underscores the importance of including EMH in the differential diagnosis for spinal cord compression in patients with chronic anaemia and highlights the critical role of early diagnosis and surgical intervention for preventing permanent neurological deficits.
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