Spinal cord compression due to extramedullary hematopoiesis in patient with Beta thalassemia major

Abstract

we reported an18 years old male with beta thalassemia major who presented with back pain progressive over 1 month associated with numbness in thighs, together with lower limbs weakness and difficulty walking. he also mentioned having difficulty in passing urine. he received radiotherapy which results in significant improvement in symptoms

Protocol for extramedullary hematopoiesis in patients with transfusion‐dependent β‐thalassemia (TDT): A systematic review

“…In our academic institute, we encountered two patients, in different timelines, with EMH in BTM. Both patients were managed by radiotherapy with excellent outcomes 6,10 . Our experience prompted us to explore the available literature on the management and outcomes of EMH in patients with TDT.…”

“…However, analysis of the limited retrospective studies and multiple case reports suggests that outcomes in patients managed for EMH in TDT are good. Although there is no agreed‐upon treatment guideline in such patients, various modalities have an excellent response, including conservative management such as blood transfusions and treatments such as radiotherapy and surgical decompression 6 . This systematic review will focus on combining the reported prevalence, demographics, clinical course, management, and outcomes in these patients in an attempt to provide better evidence to formulate clear management guidelines.…”

“…EMH is a condition in which blood production occurs outside the physiological domain, the bone marrow. EMH can occur in various organs, including the spleen, liver, lymph nodes, thymus, heart, breasts, prostate, kidneys, adrenal glands, pleura, retroperitoneal tissue, skin, peripheral and cranial nerves, and the spinal cord 6 . EMH is more common in NTDT (20%) compared with TDT (1%) 7 .…”

“…Due to its rare occurrence, data related to EMH in TDT are scattered and need compilation to provide cumulative evidence on its demographics, clinical course, and more so, its management and outcomes. There is at least one retrospective study and multiple case reports on EMH in BTM with variable management strategies and outcomes 6‐8,10 . We have conducted a thorough literature search and identified a total of 254 patients with TDT and EMH.…”

“…EMH can occur in various organs, including the spleen, liver, lymph nodes, thymus, heart, breasts, prostate, kidneys, adrenal glands, pleura, retroperitoneal tissue, skin, peripheral and cranial nerves, and the spinal cord. 6 EMH is more common in NTDT (20%) compared with TDT (1%). 7 However, the prevalence of EMH as high as around 13% has been reported in TDT patients in one study.…”