Thoracic cord compression by extramedullary hematopoiesis in thalassemia

Abstract

Extramedullary hematopoiesis (EMH) refers to hematopoiesis occurring outside the medulla of bone. It may be physiologic or due to pathological conditions like hematopoietic disorders. EMH can involve liver, spleen, thorax, and lymph nodes. It can involve paraspinal tissues with extension and involvement of spinal canal. In our case, the diagnosis was confirmed by the history of the patient stating underlying hematological condition and by magnetic resonance imaging (MRI) findings showing large soft tissue masses in paraspinal areas with involvement of spinal canal and leading to cord compression.

Thalassemia, extramedullary hematopoiesis, and spinal cord compression: A case report

“…The usual location of compression is the thoracic spine due to a currently unidentified mechanism, but certain theories have been suggested. [ 12 21 ] Being a relatively rare cause of spinal cord compression, management guidelines do not exist. However, treatments that have been tried and found to be effective are hydroxyurea, hyper transfusion, radiotherapy, surgical decompressive laminectomy, or a combination of these[ 7 8 13 19 ] and around half of all patients presenting with cord compression will improve on blood transfusions alone.…”

Discontiguous epidural lesions in a much transfused patient

“…Extramedullary hematopoiesis in adults is a compensatory response[ 1 2 ] resulting from failure of adequate production of myeloid precursors in the bone marrow and is commonly found in hemoglobinopathies and myeloproliferative disorders. [ 2 3 ] It commonly occurs in the liver, spleen, thorax, and lymph nodes.…”

“…Extramedullary hematopoiesis in adults is a compensatory response[ 1 2 ] resulting from failure of adequate production of myeloid precursors in the bone marrow and is commonly found in hemoglobinopathies and myeloproliferative disorders. [ 2 3 ] It commonly occurs in the liver, spleen, thorax, and lymph nodes. [ 2 3 ] 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 3 ] It commonly occurs in the liver, spleen, thorax, and lymph nodes. [ 2 3 ] 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[ 1 2 3 ] (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.…”