Case presentation
An 18 years old male known to have beta thalassemia major on regular blood transfusion every 3 weeks (figure 1). he is on deferasirox (iron chelator agent) but he is not compliant with it and oral hydroxyurea 1000 mg daily. presented to the emergency department with history of mid thoracic to lower back pain progressive over 1 month associated with numbness in his both thighs, together with lower limbs weakness and difficulty in walking. he also mentions having difficulty in passing urine. He denies any other symptoms. And no history of trauma.
His labs showed hemoglobin 8.6 gm/dl, leukocytes count and platelet were normal. his bilirubin total was 80.9 umol /l and direct bilirubin 13.2 umol/L, ALT 56.2 U /L and ALT 64 U/L, and ferritin 2345 mcg/L, other labs unremarkable (table 1). on examination he had spastic gait, lower limb examination showed hyperreflexia with positive ankle clonus, plantar upgoing bilaterally, strength in hip flexion was 4/5 bilaterally otherwise 5/5 for the remaining muscles group, absent vibration and decrease sensation in lower limbs with no specific dermatome, mild spasticity more in left leg , PR showed decrease sensation and normal tone. cranial nerves and upper limb examination both were normal
MRI spine showed intraspinal posterior extramedullary epidural lobulated lesions extending from lower border of T2 vertebral body up to T9 vertebral body. They demonstrate immediate to low T1WI signal intensity and dark T2WI signal intensity with mild heterogenous postcontrast enhancement (figure 2, A, B). They are causing moderate to severe spinal canal stenosis and significant compression and anterior displacement as well as thinning of the spinal cord. There is intermedullary high T2WI signal intensity at the compressed segment of the spinal cord suggesting edema and/or myelomalacia. Similar intraspinal anterior epidural lesions are seen at T7 and T10 levels and seen extending through bilateral exiting neural foramina; left more than right (figure 2, D, E). Similar anterior intraspinal lesions are seen at L5-S1 level (figure 2, C) demonstrating interval increase in size, as compared to previous MRI lumbar spine done two years ago compromising the thecal sac. Impression was intraspinal epidural lobulated lesions causing significant neural compromise, the appearances are highly suggestive of extramedullary hematopoiesis.
Patient seen by neurosurgery team and they advise for radiotherapy before any surgical intervention for the follow reasons ; the extramedullary hematopoiesis tissue is radiosensitive , beside that the lesion involves long segment of spinal column (almost from T2-T9) so surgery will involve bone removal (hemilaminectomy ) in all levels and may affect stability of spinal column , lastly the Patient has weak fragile bones related to his general condition and In case of instrumentation and fusion , there is high probability of failure , and they suggest that In case of failure of radiotherapy , they can offer the surgical option to the patient with major risks due to above mentioned reasons .
Oncology radiotherapy saw the patient and decided to proceed to radiotherapy. patient admitted to hospital started on intravenous dexamethasone waiting for the radiotherapy, he received blood transfusion as well to improve his hemoglobin from 7.5 to 11.2 gm /dl. Later on, admission he received ten session of radiotherapy with significant improvement in his weakness and other neurological symptoms.