Compressive Dorsal Myelopathy Secondary to Extramedullary Hematopoiesis in a Thalassemic Patient

Abstract

Background Extramedullary hematopoiesis (EMH) is one of the rare causes of spinal cord compression (SCC). It results from noncancerous proliferation of hematopoietic tissue outside the bone marrow as a compensatory mechanism for ineffective erythropoiesis. It occurs in the paraspinal area in 11–15% of thalassemic patients in intermediate and severe cases causing a paraspinal compressive mass. We present a rare case of spinal EMH with thoracic cord compression in a 22-year-old female with beta thalassemia who presented with paraparesis and we provide a review of literature. Case Report A 22-year-old female patient with a known history of beta thalassemia presented with subacute onset of weakness and numbness of both lower limbs with a sensory level at T6. Magnetic resonance imaging (MRI) of the dorsal spine showed cord compression secondary to paraspinal EMH from T2 to T9 with most prominent compression over T5. She was managed with blood transfusion and low-dose radiotherapy. Conclusion Although rare, EMH should be suspected in thalassemic patients presenting with paraplegia. Treatment with blood transfusions is usually effective. Other options include radiotherapy, surgery, hydroxyurea or a combination of these modalities.

Epidural myelolipoma in a Silken Windhound

“…5 There are multiple reports of spinal cord compression due to extramedullary hematopoietic tumors in people afflicted with severe anemia, most commonly attributed to beta-thalassemia. [13][14][15][16] Similar findings were recently reported in a domestic shorthaired cat with chronic nonregenerative anemia and probable myelodysplasia. 17 In contrast, myelolipomas are not associated with hematologic disorders and tend to occur in significantly older patients.…”

“…In people, extramedullary hematopoietic tumors are typically located within the mediastinum or epidural space 5 . There are multiple reports of spinal cord compression due to extramedullary hematopoietic tumors in people afflicted with severe anemia, most commonly attributed to beta‐thalassemia 13–16 . Similar findings were recently reported in a domestic shorthaired cat with chronic nonregenerative anemia and probable myelodysplasia 17 .…”

Thalassemia in the emergency department: special considerations for a rare disease

“…Treatment includes intravenous glucocorticoids, surgical Fig. 3 Management of the patient with thalassemia presenting to the ED with abdominal pain decompression, radiation therapy, and blood transfusion to alleviate the hypoxic stimulus for extramedullary hematopoiesis [66,67]. In the absence of focal neurological deficits, considering acute or chronic vertebral fractures is important as patients with thalassemia are at an increased risk of osteoporosis [68][69][70][71].…”