Soft Tissue, Extramedullary hematopoiesis [LMP93820]

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Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Tissue Renewal, Regeneration, and Repair
Diagnosis: 
Extramedullary hematopoiesis
Species: 
Human
Organ System: 
Bones, Joints, and Soft-Tissue Tumours
Gender: 
Male
Age: 
22 years
Diagnosis Code: 
ICD-10: M79.9
Slide Contributor Information
Gao, A., Kiehl, TR.

Keywords

EMH, thalassemia

Clinical History

22-year-old man with thalassemia intermedia presents with thoracic myelopathy and a paraspinal soft tissue mass lesion.

Clinical Discussion

Extramedullary hematopoiesis (EMH) is a physiologic condition in the fetal state and can occur in adults as a compensatory phenomenon secondary to failure of the bone marrow to produce adequate blood elements. While liver, spleen, and lymph nodes are common sites, the CNS and its surrounding structures are rarely involved by EMH. Microscopically, there is trilineage hematopoiesis with an infiltrate of erythroid and myeloid elements and megakaryocytes. The most common underlying hematologic conditions associated with CNS EMH are beta-thalassemia and myelofibrosis.