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The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology · 2025

A rare case of extramedullary hematopoiesis: when the pericardium produces blood cells.

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Hayar Salah-Eddine, Tamir Mehdi, Khaldi Mohamed, Bouziane Maha, Haboub Meryem, Arous Salim, Bennouna Mohamed Ghali, Drighil Abdenasser, Habbal Rachida, Mellouki Abderrahmane

Аннотация

BACKGROUND: Cardiac tamponade is an extreme cardiological emergency, fatal in the absence of rapid intervention. This case report highlights a noteworthy and rare correlation between post-polycythemia vera myelofibrosis and extramedullary hematopoiesis affecting the pericardium, leading to tamponade or pericardial effusion.

CASE PRESENTATION: A 69-year-old female with a history of polycythemia vera presented with worsening dyspnea, fever, and altered condition. Examination revealed low blood pressure, tachycardia, jugular vein distention, and muffled heart sounds, leading to a diagnosis of cardiac tamponade due to a large pericardial effusion. Emergency pericardiocentesis was performed, revealing serosanguineous fluid with signs of clonally proliferative hematopoietic cells, indicating possible progression to myelofibrosis. Bone marrow biopsy confirmed post-polycythemia vera myelofibrosis. The patient's condition improved, and she was referred back to her hematologist for further management.

CONCLUSION: Increased awareness may improve early diagnosis and treatment, ultimately enhancing patient outcomes.