Discontiguous Epidural Lesions in a Much Transfused Patient.

Sir,

An 18-year-old male patient presented with mild weakness of bilateral lower limbs and walking difficulty for 1-month duration. He was previously diagnosed to be afflicted by beta thalassemia and had a history of multiple blood transfusions. On examination, he was found to be mildly icteric, had hepatosplenomegaly, and had a hemoglobin level of 4.5 g%. His total counts were normal. Magnetic resonance imaging (MRI) of the spine showed discontiguous epidural mass isointense (to the spinal cord) on both T1 and T2 sequences, causing thecal sac and cord compression at cervical, dorsal, and lumbar levels without any vertebral destruction [Figure 1]. In addition, there was extensive paraspinal soft tissue along the thoracic spine with destruction of rib heads as well as a posterior mediastinal mass encasing the aorta [Figure 2]. Computed tomography (CT)-guided fine needle aspiration cytology of the paravertebral mass showed picture-like bone marrow with reversal of myeloid–erythroid ratio with preponderance of erythroid precursors on May–Grünwald–Giemsa staining [Figure 2]. Extramedullary hematopoiesis was diagnosed. He was transfused with four units of blood following which his power improved.

Figure 1

Sagittal T2-weighted MRI scans showing (A)cervicodorsal, (B) mid-dorsal, and (C) lower lumbar isointense epidural mass compressing thecal sac and spinal cord with no vertebral body destruction

Figure 2

(A) Coronal T1-weighted magnetic resonance scans showing paraspinal soft-tissue mass (blue arrows) at the dorsal level with splenomegaly (red star); (B) posterior mediastinal mass encasing the aorta (blue arrow); (C) soft-tissue mass destroying the rib head (blue arrows) with epidural mass pushing the cord anteriorly and to the left; and (D) erythroid precursors and reversal of the myeloid–erythroid ratio seen on May–Grünwald–Giemsa stain of the CT-guided fine-needle aspirate from the paravertebral tissue

Extramedullary hematopoiesis in adults is a compensatory response[12] resulting from failure of adequate production of myeloid precursors in the bone marrow and is commonly found in hemoglobinopathies and myeloproliferative disorders.[23] It commonly occurs in the liver, spleen, thorax, and lymph nodes.[23] Epidural involvement (as seen in this case) may be due to either extension from paraspinal tissues[2] or following stimulation of epidural embryonic cell rests or extension from vertebral marrow.[2]

Possible treatment options include surgery, radiotherapy, chemotherapy, and hypertransfusion[123] (as the lesions diminish or even disappear on when the patient receives blood). The last alternative is suitable in patients like ours with discontiguous, multiple lesions and minimal deficits.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.