Abstract
Extramedullary hematopoiesis (EMH) occurs commonly in patients with severe thalassemia who receive inadequate treatment. The diagnosis of thalassemia intermedia is purely clinical and is usually made after a period of observation to determine that the patient is able to maintain a satisfactory hemoglobin (Hb) level for a long time without the need for blood transfusions. The incidence of EMH in patients with thalassemia intermedia may reach up to 20%, compared to polytransfused thalassemia major patients where the incidence remains <1% [1]. Besides the usual regions of hematopoiesis, blood cells can be formed in unusual sites like the liver, spleen and lymph nodes to meet the demands of hematopoiesis [2]. There are a few reports where EMH has involved some rare places such as the perirenal and paravertebral regions, paranasal sinuses, clivus, meninges, spinal epidural spaces, prostate, adrenals, pleura, breast, thymus, kidney, sweat gland, broad ligament and retroperitoneal space [2]. This unusual phenomenon, especially when it involves the spinal cord can lead to neurological deficits [3]. A paraspinal location for the hematopoietic tissue occurs in 11-15% of cases with EMH [1]. Abstract
Highlights
Extramedullary hematopoiesis (EMH) occurs commonly in patients with severe thalassemia who receive inadequate treatment
Spinal cord compression due to EMH has been reported as an uncommon complication in intermedia or major beta-thalassemia patients occurring mostly in children or young adults
The masses represent extrusion of the marrow through the thinned cortex of the posterior ribs. It usually has a predilection for the lower thoracic spine where the limited mobility and narrow spinal canal predisposes itself to spinal cord compression [4]
Summary
This case report showed that rare, a paravertebral EMH should always be considered in the differential diagnosis of any upper motor neuron disease condition in patients with thalassemia and appropriate studies performed to investigate the probable paravertebral ectopic marrow. We conclude that drug therapy may be the optimal therapeutic approach in such cases with mild paraparesis. Physicians should be taken into account the diagnosis of a congenital anemia when evaluating neurological symptoms. There is not standard care in these cases and therapeutical alternatives such us radiotherapy, surgery, hyper-transfusion or hydroxyurea must be chosen in a case-by case- manner
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