Extramedullary haematopoiesis presenting with cardiac tamponade in a patient with polycythaemia vera

BMJ Case Rep. 2017 Aug 10;2017:bcr2017221316. doi: 10.1136/bcr-2017-221316.


A 71-year-old man with a history of polycythaemia vera, diagnosed 4 years ago, presented to the emergency room with shortness of breath. A bedside echocardiogram revealed a large pericardial effusion with features concerning for pericardial tamponade. A left anterior thoracotomy and a pericardial window were emergently performed in the operating room and relieved the patient's symptoms. Histology evaluation of the pericardial fragments and pericardial fluid revealed the presence of trilineage haematopoietic elements without any increase in the blasts. A bone marrow core biopsy revealed an increase in reticulin fibre and increase in the number of blasts of 5%-10%, whereas peripheral blood testing was positive for JAK2 V617F mutation. This case report reviews the literature for cases of extramedullary haematopoiesis associated with myeloproliferative neoplasms.

Keywords: haematology (incl blood transfusion); heart failure.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Cardiac Tamponade / etiology*
  • Cardiac Tamponade / surgery
  • Diagnosis, Differential
  • Echocardiography
  • Hematopoiesis, Extramedullary*
  • Humans
  • Male
  • Pericardium / pathology*
  • Polycythemia Vera*
  • Primary Myelofibrosis / complications
  • Primary Myelofibrosis / diagnosis*
  • Primary Myelofibrosis / pathology