Recurrent thoracic cord compression due to extramedullary haematopoiesis in thalassaemic patients–A case presentation and literature review

Abstract

Extramedullary haematopoesis (EMH) in thalassaemic patients to result in spinal cord compression is a rare phenomenon. EMH occurs as compensatory sequelae in those with chronic anaemia such as thalassaemia, other congenital haemolytic anaemia, haemoglobinopathies, and myelofibrosis. Common sites of EMH include liver, spleen, kidneys and lymph nodes, but rarely the paravertebral and spinal cord regions. In our literature review, it was found that only case reports and case series have been published. We present a case of recurrent thoracic spinal cord compression caused by EMH in a 32-year-old thalassaemic Chinese man treated with surgical decompression and spinal stabilisation. The key to successful treatment is prompt diagnosis with early clinical suspicion, magnetic resonance imaging and individualised treatment for each patient. Surgical decompression provides immediate neural decompression while subsequent anaemia management may reduce the risk of recurrence. In the present case, a combination therapy of blood transfusion, surgical decompression and radiotherapy has been shown to deliver successful outcomes in such cases.

Recurrent spinal cord compression due to extramedullary hematopoiesis in thalassemia patient

“…Unfortunately, radiotherapy also carries risks, including possible deterioration of neurologic function due to tissue edema, bone marrow suppression, and long-term side effect of radiotherapy. [ 14 ] Additionally, although many cases reported complete resolution of symptoms within days of starting radiotherapy, several cases, including our case, showed recurrence. The reported recurrence rates in the literature range between 19% and 37%.…”

“…Since its description by Gatto et al in 1954, [13] only a handful of case reports and series have been reported in the literature. It usually has a predilection for the lower thoracic spine where the limited mobility and narrow spinal canal predispose itself to spinal cord compression [14,15] . The clinical presentation may mimic paravertebral tumors, including back pain, paraparesis, paresthesia in the lower limbs, gait instability, and sphincter disturbances [16] .…”

“…Disadvantages of surgical option include risk of bleeding associated with the high vascularity of the mass, risks of operating on anemic individuals who are predisposed to shock, incomplete excision in cases of diffuse involvement, [32] and instability of the spine after multi-levels laminectomy [33] . Unfortunately, recurrence was also seen after decompression surgery [14] . After all, the lack of treatment guidelines presents a challenge in treating such conditions with no modality of treatment that has been proven superior to another.…”

“…It usually has a predilection for the lower thoracic spine where the limited mobility and narrow spinal canal predispose itself to spinal cord compression. [ 14 , 15 ] The clinical presentation may mimic paravertebral tumors, including back pain, paraparesis, paresthesia in the lower limbs, gait instability, and sphincter disturbances. [ 16 ] Complete paraplegia of the lower limbs has also been reported.…”

“…In order to halt the production of overgrown marrow tissue, radiation therapy has been used in many cases of EMH mass either as monotherapy [ 15 , 17 , 25 , 26 ] or combined with surgery [ 14 , 27 , 28 ] with good results. Some authors even suggested that radiation therapy should be the primary treatment modality with surgical intervention reserved for recurrent cases postradiation.…”