Spinal Cord Compression Secondary to Extramedullary Hematopoiesis: Case Report and Review of the Literature

Abstract

Extramedullary hematopoiesis (EMH) is a rare cause of spinal cord compression (SCC). EMH represents the growth of blood cells outside of the bone marrow and occurs in a variety of hematologic illnesses, including various types of anemia and myeloproliferative disorders. Although EMH usually occurs in the liver, spleen, and lymph nodes, it may also occur within the spinal canal. When this occurs, the mass effect can compress the spinal cord, potentially leading to the development of neurological deficits. We present a case of SCC secondary to EMH. This report illustrates the importance of considering EMH in the differential diagnosis of SCC, even in the absence of signs of its most common etiologies.

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

“…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].…”

Spinal Cord Compression due to Extra Medullary Hematopoiesis in a Case Of Thalassemia Minor

“…[1,[3][4][5] EMH is commonly seen in organs involved in hematopoiesis in fetal life like liver or in organs of reticuloendothelial system like spleen and lymph nodes. However it is also known to occur in other organs like pleura, adrenals, breast, thymus, kidneys, gastrointestinal tract, intracranial structures, retroperitoneal tissue, sweat glands, prostate, broad ligaments, peripheral nerves, heart, retroperitoneal fat, Para spinal regions.…”

“…However it is also known to occur in other organs like pleura, adrenals, breast, thymus, kidneys, gastrointestinal tract, intracranial structures, retroperitoneal tissue, sweat glands, prostate, broad ligaments, peripheral nerves, heart, retroperitoneal fat, Para spinal regions. [1,2,5] . Para spinal location of EMH has been noted in 11-15% cases.…”

“…Apart from physiological conditions like during fetal development, EMH occurs as a compensatory phenomenon in conditions in which there is generally a state of chronic anemia including Thalassemia, among other pathologies. [1] EMH is mainly seen in liver, spleen and lymph nodes, pertaining to their hematopoietic activity during fetal development, but also rarely occurs in spinal region. The presence of EMH is particularly worrisome in the spinal region as it acts as a space occupying lesion (SOL) in a region that is already compromised for space, thus producing morbid consequences.…”

“…[2] Due to rarity of this entity, there is no definite consensus regarding the treatment for this condition which includes several options like surgical resection, Radiotherapy(RT), Hypertransfusion (HT), Hydroxyurea (HU) administration or a combination of the above. [1 ] We present a case of a 43 year old male who presented with low backache, bilateral lower limb weakness and icterus in whom EMH was suspected on intraoperative squash preparation and subsequently proven on histopathology and immunohistochemistry (IHC).…”