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.