Abstract

We report a case of a man in his early 60s presenting with bilateral adrenal and pulmonary haemorrhages as an initial presentation of polycythaemia vera (PV). Symptomatology included severe compressive chest pain radiating to epigastrium, with unremarkable physical findings, parameters and ECG. Blood investigations showed an elevated haemoglobin (174 g/L, reference range (RR): 141-172g/L) and haematocrit (55.7%, RR: 40.4%–50.4%) levels.

Cross-sectional imaging excluded aortic dissection, but imaging repeated 48 hours after his admission for acute dyspnoea and worsening abdominal pain showed bilateral alveolar and adrenal haemorrhages. Cortisol level was 27 nmol/L (RR: 145–619 nmol/L). Investigations confirming PV included the presence of a Janus kinase 2 (JAK2V617F) gene mutation, hypercellularity with erythroid hyperplasia on bone marrow microscopy and a low serum erythropoietin (2.6 mIU/mL, RR: 4.3–29.0 mIU/mL). Aspirin, hydroxyurea, venesection and cortisol replacement were initiated to get good treatment outcome.