Unraveling a Rare Case of Epidural Extramedullary Hematopoiesis in a Patient With Transfusion-Dependent Beta Thalassemia Presenting With Spinal Cord Compression.

Thalassemia is known to induce extramedullary hematopoiesis (EMH), which is a compensatory mechanism in which the body forms blood cells outside the bone marrow. While EMH typically affects organs such as the spleen and liver, there are rare instances where it leads to spinal cord compression (SCC) in the epidural space. A 31-year-old male patient with transfusion-dependent beta thalassemia presented with numbness and bilateral limb weakness due to EMH. Neurological examination revealed increased tone in both legs, reduced power, loss of crude touch and pain sensation, and increased deep tendon reflexes. Magnetic resonance imaging (MRI) indicated a lobulated soft tissue structure in the posterior dural intrathecal space causing SCC. Laminectomy of the T2-T8 vertebrae was done, after which the lesion was identified and completely removed. Post-surgery, significant neurological improvements were observed in both motor and sensory functions. Thalassemia patients presenting with symptoms of SCC should be investigated for the presence of epidural EMH. Treatment options include decompressive surgery, blood transfusions, hydroxyurea, and radiotherapy.

Distributions and burden of coronary calcium in asymptomatic Saudi patients referred to computed tomography.

BACKGROUND: Unlike Western and Asian populations, the prevalence and severity of coronary artery calcification (CAC) have not been adequately examined in Saudi Arabia and other nearby Arab Gulf countries. OBJECTIVES: To estimate the age and gender specific percentiles of coronary calcium score (CCS) and to study the severity of CAC in relation to patient risk in a large sample of asymptomatic Saudi patients. METHODS: Retrospective cross-sectional study was conducted between July 2007 and December 2017 at a large Cardiac Centre in Riyadh, Saudi Arabia. The target was adult patients without pre-existing CAD referred to (64 multidetector spiral) computed tomography for standard indications. RESULTS: A total 2863 patients were included in the current analysis. The 90th percentile of CCS was 95.0 in males compared with 53.2 in females and was 823.95 in patients aged ≥ 75 years compared with zero in patients < 40 years. Extensive CAC (CCS > 400) were 3.1% in males compared with 1.6% in females and 14.0% in patients aged ≥ 75 years compared with 0.0% in patients < 40 years. CCS was steadily higher with increasing European systematic coronary risk evaluation; 3.1 ± 22.5 in mild risk, 37.1 ± 201.9 in moderate risk, 116.1 ± 256.1 in high risk, and 131.0 ± 222.0 in very high risk. CONCLUSIONS: As expected, the findings confirm the higher burden of CAC in males, older age, and higher CAD risk. The burden of CAC in current patients is much lower than reported in US and other Western patients. Local cardiologist should consider using local rather than US percentiles of CCS.