Complete recovery from paraparesis in spinal cord compression due to extramedullary haemopoiesis in beta‐thalassaemia by emergency radiation therapy

Abstract

Extramedullary haemopoiesis (EMH) is a complication commonly associated with beta-thalassaemia intermedia; it is frequently asymptomatic but can sometimes lead to symptomatic tumour-like masses. No guidelines or common consensus are available in literature regarding the different treatment strategies and only single cases have been reported. We describe a case of spinal cord compression due to intrathoracic EMH masses treated with combined radiotherapy and hydroxyurea.

Incomplete paraplegia caused by extramedullary hematopoiesis in a patient with thalassemia intermedia

“…10 Tissue edema and pancytopenia resulting from this therapy might worsen the neurological symptoms. Redda et al 8 reported no acute toxicity using total radiotherapy dose of 10 Gy in five sessions of 2 Gy per day. They also reported significant pain relief after first radiotherapy fraction.…”

“…6 As paraspinal EMH is a rare condition, evidence-based therapy from randomized control trials is lacking. 7,8 Most of the reported treatment options are based on case reports and Incomplete paraplegia in thalassemia intermedia N Hisamud-din et al small case series. 8 Management options include hypertransfusion, radiotherapy, surgical decompression, hydroxyurea or a combination of these modalities.…”

“…7,8 Most of the reported treatment options are based on case reports and Incomplete paraplegia in thalassemia intermedia N Hisamud-din et al small case series. 8 Management options include hypertransfusion, radiotherapy, surgical decompression, hydroxyurea or a combination of these modalities. Treatment should be individualized as each treatment option carries its own advantages and disadvantages.…”

“…Redda et al observed improvement in hematological parameters after 6 months of hydroxyurea therapy. 8 In conclusion, a prompt recognition of the condition based on clinical presentation and typical radiological findings should be made. Early treatment is recommended to prevent irreversible damage to the spinal cord.…”

Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia

“…MRI ( Figures 1, 2, 3 and 4) showed vertebral canal stenosis with spinal cord compression. This finding illustrates a rare complication of thalassemia, an extramedullary hematopoietic center due to ineffective erythropoiesis, causing vertebral bone hyperplasia 1,4,5 . Given the substantial number of segments affected, surgical procedures were contraindicated based upon hemorrhage risk.…”