A case report of extramedullary haematopoeisis in lumbosacral region presenting as cauda equina syndrome

Abstract

Introduction: Extramedullary hematopoeisis (EMH) is defined as formation of blood cells outside the bone marrow. It occurs most commonly in the liver and spleen in patients having disorders that lead to chronic anaemia. EMH in spinal canal is a very rare site and cauda equina syndrome due to EMH has very few cases presented in literature.

Case presentation: A 28 year old male patient presented with complain of incontinenance of bladder and bowel along with saddle anaesthesia from 10 days. Patient was a known case of beta-thalassemia intermedia. And MRI scan of the spine showed multiple well circumscribed, enhancing lesions in the epidural space extending from L5 to S3 and resulting in compression of the cauda equina. Patient underwent posterior neural decompression by a laminectomy from L5 to S3. At 3 months follow up patient had partial recovery of his bladder control and complete recovery of sensation.

Conclusion: EMH should be recognized early on the basis of clinical features and MRI findings. The various modalities available for treatment of such cases includes blood transfusion, low dose radiotherapy, hydroxyurea and surgical decompression. There are very few cases noted in the literature of such phenomenon in the lumbosacral spine. In cases of acute presentations like cauda equine surgical decompression is a treatment modality of choice.

Keywords: compression; extramedullary; hematopoiesis; thalassemia.

Figure 1

MRI T2 image of the lumbar spine showing the extramedullary masses in the anterior epidural space at L5-S3.

Figure 2

MRI T1 weighted image showing the hypointense extramedullary masses most prominent behind the S1.

Figure 3

Axial T2 weighted MRI at the level of S1 showing the anterior epidural space encroached by the extramedullary masses leading to compression.

Figure 4

Per operative images after laminectomy with the thick white arrow showing the thecal sac and the thin dark arrow showing the anterior hypervascular masses.

Figure 5

Histological examination showing hyperplastic bone marrow with many deposits of foamy histiocytes within an erythroid hyperplasia.