Case Presentation: A 74-year-old male with past medical history of hypertension, and prior tobacco abuse presented with complaints of left sided abdominal pain for one day. He denied any chest pain, shortness of breath, or any other symptoms. The patient quit smoking 14 years ago, drank alcohol weekly and denied illicit drug use. Family history was negative for malignancy or clotting disorders. Initial vitals showed hypoxia, with oxygen saturation of 87% on room air. The remaining vitals were within normal limits. Respiratory and cardiovascular exam were unremarkable. Abdominal examination showed left-sided abdominal tenderness to palpation with no guarding or rebound, and normal bowel sounds. A complete blood count showed a hemoglobin of 18.1 g/dL, hematocrit of 53.6%, white blood count of 13,100/µL, platelet count of 220,000/µL and red blood cell count of 5410/µL. Previous hemoglobin values had ranged between 15.0 g/dL to 16.7 g/dL over the past few years, with no hematological workup. His borderline erythrocytosis was attributed to his history of smoking. BNP was elevated at 4275 pg/mL, and troponin was negative. CT Abdomen showed an acute left renal infract with occluded distal left renal artery with left iliac artery aneurysm containing mural thrombus. It was also concerning for possible segmental pulmonary emboli in bilateral lower lobes of the lungs. A subsequent CT chest showed sub-massive saddle pulmonary embolism with right heart strain. He was also found to have a non-occlusive right femoral deep vein thrombosis. The patient was started on a heparin drip and underwent successful catheter directed thrombolysis. He was then transitioned to Warfarin 7.5mg daily and discharged with outpatient follow-up. The patient was admitted six months later with hypoxia, concerning for another embolism. INR was therapeutic at 2.2. Repeat CTPA showed increased clot burden, but resolution of the saddle embolus and new small subsegmental emboli. Hematology was consulted for persistently elevated hemoglobin. Further workup included erythropoietin levels as well as BCR-ABL, JAK2, MPL and CALR to rule out myeloproliferative disorders. The patient was found to have a positive JAK 2 V617F and exon 12-15, confirming polycythemia vera. The patient was started on Aspirin and hydroxyurea. He was also treated with phlebotomy, as needed, to maintain Hct < 45%, requiring it almost monthly. At one year follow-up, patient was noted to be doing well.
Discussion: Polycythemia vera, a rare myeloproliferative disorder characterized by the overproduction of red blood cells, is associated with high risk of thromboembolic events. Our patient had recurrent pulmonary emboli due to polycythemia vera. The risk of thromboembolic events can be minimized by maintaining the hematocrit < 45%. Our patient did not have any thromboembolic events after treatment with hydroxyurea, aspirin and phlebotomy. Our case highlights the importance of recognizing elevated blood counts and investigating for potential causes in a timely manner. Physicians should be careful in avoiding anchoring when evaluating patients with erythrocytosis. Our patient’s erythrocytosis was blamed on his prior tobacco abuse until prior to his admission.
Conclusions: We present the case of patient who developed recurrent pulmonary emboli secondary to polycythemia vera. We highlight the need for timely diagnosis of polycythemia vera and early workup for polycythemia vera in patients with borderline erythrocytosis.