Lumbosacral extradural extramedullary hematopoiesis in thalassemia major causing spinal canal stenosis

Lumbosacral extradural extramedullary hematopoiesis in thalassemia major causing spinal canal stenosis:




Lumbosacral extradural extramedullary hematopoiesis in thalassemia major causing spinal canal stenosis




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Sumatha Channapatna Suresh1, Bharath Raju2, Fareed Jumah2, Anil Nanda2
Department of Rheumatology, St. John’s Medical College and Hospital, Bengaluru, Karnataka, India,
Department of Neurosurgery, Rutgers Robert Wood Johnson University Hospital, New Brunswick, New Jersey, United States.

Correspondence Address:
Bharath Raju
Department of Neurosurgery, Rutgers Robert Wood Johnson University Hospital, New Brunswick, New Jersey, United States.

DOI:10.25259/SNI_563_2020

Copyright: © 2020 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.


How to cite this article: Sumatha Channapatna Suresh1, Bharath Raju2, Fareed Jumah2, Anil Nanda2. Lumbosacral extradural extramedullary hematopoiesis in thalassemia major causing spinal canal stenosis. 08-Oct-2020;11:331


How to cite this URL: Sumatha Channapatna Suresh1, Bharath Raju2, Fareed Jumah2, Anil Nanda2. Lumbosacral extradural extramedullary hematopoiesis in thalassemia major causing spinal canal stenosis. 08-Oct-2020;11:331. Available from: https://surgicalneurologyint.com/surgicalint-articles/10318/


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Date of Submission
22-Aug-2020

Date of Acceptance
17-Sep-2020

Date of Web Publication
08-Oct-2020

Abstract



Background: Extramedullary hematopoiesis (EH) is common in patients with ineffective erythropoiesis like thalassemia major (TM). EH commonly involves intra-abdominal organs (e.g., spleen and liver), but rarely involves vertebral bodies. Here, we reviewed images of EH contributing to spinal canal stenosis.

Case Description: A 19-year-old male with beta-thalassemia major (TM) presented with pain and bilateral lower extremity neurogenic claudication. Bilaterally, on examination, he had positive straight leg raising to 30°, a loss of the Achilles responses, and decreased pain appreciation in the L5S1 distributions. The lumbar MR showed anterior epidural lobulated mass lesions at L5 and S1, contributing to marked canal stenosis. Following an L5/S1 laminectomy for decompression, the biopsy revealed extramedullary hematopoietic tissue.

Conclusion: Patients presenting with the lower extremity symptoms/signs, ranging from low back pain to neurogenic claudication and even paraplegia, may have EH secondary to TM. Treatment options include hypertransfusion, local radiation therapy, and/or surgical decompression.

Keywords: Beta-thalassemia, Extramedullary hematopoiesis, Radiculopathy, Spinal stenosis

INTRODUCTION



Extramedullary hematopoiesis (EH) is observed in many hemoglobinopathies, including beta-thalassemia major (TM). Here, we report a patient with TM who, while receiving chronic transfusions, developed epidural EH resulting in spinal canal stenosis at the L5/S1 level requiring operative decompression.
CASE DESCRIPTION



A 19-year-old male with beta-TM complicated by chronic iron overload, presented with bilateral lower extremity pain, numbness, and neurogenic claudication. Bilaterally, on examination, he had positive straight leg raising to 30°, a loss of the Achilles responses, and decreased sensation in the L5-S1 distributions. When the lumbar MR demonstrated L5S1 EH responsible for spinal canal stenosis [ Figure 1 ], the patient underwent an L5S1 laminectomy; postoperatively, all preoperative symptoms and signs resolved. The histopathological evaluation of the EH mass revealed hyperplastic hematopoietic tissue.






Figure 1:

(a and b) Sagittal T2-weighted images showing L5, S1 level anterior epidural hypointense lobulated mass lesion attached to the posterior surface of the vertebral body with severe canal stenosis. (c and d) Axial T2-weighted images showing bi-lobulated lesion protruding from the vertebral body with near total canal stenosis.



DISCUSSION


Location and frequency of occurrence of spinal EH with TM

EH has a prevalence in transfusion-dependent beta-thalassemia of 4 , 6 ] EH typically occurs systemically (e.g., in many abdominal and/or thoracic organs). In the central nervous system (CNS), it commonly involves the choroid plexus, cranial dural folds, and peripheral nerves and can even be found within some brain tumors.[ 1 ] In the spinal canal, EH often involves contiguous vertebral bodies and can extend into the spinal canal where it may presents as a pseudotumor.[ 2 ]
Differential diagnosis and MR findings of EH with TM

The differential diagnoses for EH spinal lesions include an epidural infection, vertebral fracture, or epidural hematoma.[ 3 ] MRI, the study of choice, frequently demonstrates a lobulated hypointense mass dorsal to a vertebral body that does not enhance with contrast.
Treatment options for EH with TM

Treatment of EH with TM include; hypertransfusion, local radiation therapy, and/or surgical decompression. Blood transfusions can prevent further progression of EH pseudotumor.[ 5 ] Radiotherapy reduces the volume of pseudotumor, thus relieving local compressive symptoms/signs. Surgery, such as decompressive laminectomy, offers immediate relief of cord compression, and/or canal obstruction.[ 1 , 3 ]

Declaration of patient consent



The authors certify that they have obtained all appropriate patient consent.
Financial support and sponsorship



Nil.
Conflicts of interest



There are no conflicts of interest.

References



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