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

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.