Extramedullary hematopoiesis causing spinal cord compression in polycythemia vera: A case report and literature review

Extramedullary hematopoiesis Spinal cord compression Hepatosplenomegaly
DOI: 10.1016/j.ctro.2022.09.009 Publication Date: 2022-10-03T12:25:43Z
ABSTRACT
Extramedullary hematopoiesis (EMH) is a rare cause of spinal cord compression defined as finding hematopoietic elements outside the physiological location in bone marrow. We report case 70-year-old man with JAK 2 positive myeloproliferative syndrome type polycythemia vera (PV), initially treated hydroxyurea. Two years after diagnosis, he presented progression biological and clinical evolution associating hyperleukocytosis hepatosplenomegaly no evidence acute myeloid leukemia. Treatment hydroxyurea ruxolitinib was introduced. Six months later, symptoms suggesting from T2 region appeared. Medullary MRI revealed multistage injury to S1, while brain CT excluded any intracranial location. The biopsy diagnosed extramedullary CD34 + blast cell, corresponding vera. Given lack consensus review literature, irradiation planned treat volume T1 S2 dose 18 Gy 10 sessions 1.8 Gy. At end radiotherapy, patient started recover his motor sensory functions. walked without assistance had significant toxicity. Using radiotherapy caused by EMH justified excellent early response major side effects. present here this systematic literature on matter.
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