Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy

Abstract

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of thalassemia intermedia with paraplegia treated successfully with radiotherapy.

Spinal cord compression due to extramedullary hematopoiesis in a patient with E-beta-thalassemia managed without radiation or surgery

“…Therefore, EMH occurs in patients with thalassemia whose chronic severe anemia has not been corrected by blood transfusion. Typical sites of EMH can be any organ that participates in hematopoiesis during fetal development such as the spleen, liver, kidneys, and lymph nodes as well as other less commonly known sites such as heart, breasts, prostate, broad ligaments, pleura, cranial nerves, and the spinal canal [1,2]. Particularly, in patients with thalassemia, paraspinal involvement is seen in approximately 15% of the cases [3].…”

“…Clinical presentation of EMH elements can range from an incidental finding during the workup for the anemia, to paresthesia/paraplegia/other neurological presentations as in our patient due to spinal cord compression, hemoptysis if pulmonary tissues are involved, cardiac tamponade due to massive pericardial effusion, renal dysfunction, or mistakenly as neoplasm of any organ [1]. The diagnosis of EMH involving the paraspinal tissue/spinal cord compression is best made based upon the combination of history of thalassemia with undertreated severe anemia and isodense to hyperdense T1-T2 weighted findings on MRI with gadolinium contrast preparation [2,3]. …”

“…In review of literature, radiation therapy and/or surgery are the more commonly used treatment for spinal cord compression from EMH than either monotherapy or a combination of hypertransfusion and hydroxyurea [2–4]. Based on the review of previous reported symptomatic cases of spinal cord compression, depending on the severity of neurological deficit and spinal involvement, most were treated surgically followed by optional radiation [1].…”

“…Resection of the mass can lead to quick decompression [1]. EMH tissues are highly responsive to low-dose radiation 750–3500 cGy with reduction in volume as much as 16.4% after a treatment course [2,3]. Nonetheless, it was also reported that recurrence rate with radiation therapy or surgery can be as high as 19.1% [2], possibly due to the underlying pathophysiology of uncorrected anemia.…”

“…EMH tissues are highly responsive to low-dose radiation 750–3500 cGy with reduction in volume as much as 16.4% after a treatment course [2,3]. Nonetheless, it was also reported that recurrence rate with radiation therapy or surgery can be as high as 19.1% [2], possibly due to the underlying pathophysiology of uncorrected anemia. Cytopenia is also a concerning complication of radiation while severe bleeding can occur with surgical intervention if the volume of EMH elements is extensive as in this case.…”