Search

Extra-medullary hematopoiesis is a rare condition that corresponds to the ectopic development of hematopoietic tissue outside the bone marrow in response to bone marrow hyperstimulation. This phenomenon occurs mainly in embryonic hematopoiesis sites (spleen, liver, lymph nodes), sometimes in other organs such as the kidneys and lungs. Mediastinal localization is more rare. We present the observation of a patient in whom the diagnosis of beta-thalassemia was evoked in front of paravertebral tissue masses related to extra-medullary hematopoiesis. Observation: A 36-year-old patient hospitalized in our NYHA dyspnea department for the last three weeks, a thoracic CT scan was performed showing the presence on both sides of the dorsal spine of the 02 spontaneously dense masses enhanced after contrast. Biologically, the blood smear showed 78% erythroblastemia of Howell-Jolly’s acidophilic erythroblasts and the presence of red blood cell anisopoikylocytosis with target red blood cells and sickle cell disease. Electrophoresis of hemoglobin showed a hemoglobin F level at 90 %, we completed with molecular biology, this result is in favor of a β0 thalassemia (β0/β0 allele). Conclusion: Our observation describes an exceptional case of spinal extra-medullary hematopoiesis whose diagnosis of B-thallasemia was made on molecular biology. [ABSTRACT FROM AUTHOR]