August 2017 Imaging Case of the Month

Brandon T. Larsen, MD, PhD¹

Michael B. Gotway, MD²

Departments of Pathology¹ and Radiology²

Mayo Clinic Arizona

Scottsdale, Arizona USA

Clinical History: A 67-year-old man with a 23 pack-year history of smoking, stopping 6 years earlier, presented with a year-long history of intermittent hemoptysis consisting of small specs of blood particularly in the morning after he awoke. No sputum discoloration was reported and the patient denied shortness of breath, fever, shortness of breath, and chills. The patient also denied rash, joint pain, and night sweats. His past surgical history was remarkable only for an appendectomy, tonsillectomy, and repair of an ankle fracture, all as a young man. The patient did report some asbestos exposure in the past. He takes a multivitamin and occasional over-the counter pain relievers, but was not taking prescription medications.

Physical examination: unremarkable and the patient’s oxygen saturation was 98% on room air.

Laboratory evaluation: largely unremarkable.  Quantiferon testing for Mycobacterium tuberculosis was negative. An outside otolaryngology examination was reported to show no abnormalities. Frontal chest radiography (Figure 1) was performed.

Figure 1.  Frontal chest radiography.

Which of the following statements regarding the chest radiograph is most accurate? (Click on the correct answer to proceed to the second of nine pages)

1. The chest radiograph shows a mediastinal mass
2. The chest radiograph shows multifocal consolidation and pleural effusion
3. The chest radiograph shows multifocal smooth interlobular septal thickening
4. The chest radiograph shows a possible focal air space opacity
5. The chest radiograph shows small cavitary pulmonary nodules

Cite as: Larsen BT, Gotway MB. August 2017 imaging case of the month. Southwest J Pulm Crit Care. 2017;15(2):69-79. doi: https://doi.org/10.13175/swjpcc098-17 PDF

Medical Image of the Week: Portal Vein Thrombosis in a Patient with Polycythemia Vera

Figure 1 Panel A: CT of the abdomen demonstrating a thrombus within the portal vein (black arrow). Panel B: CT showing extension of the thrombus into the splenic vein (black arrow).

A 39-year-old man with no past medical history presented with acrocyanosis of his left second toe and right upper abdominal pain. Initial labs showed polycythemia, leukocytosis and thrombocytosis that were unchanged with intravenous fluid administration. A CT of the abdomen was obtained and showed portal vein thrombosis without evidence of cirrhosis (Figure 1). Subsequent studies revealed a positive JAK2 V617F mutation and low erythropoietin levels consistent with polycythemia vera (PV). The patient was placed on anticoagulation, low dose aspirin, and received phlebotomy.

PV is a chronic myeloproliferative neoplasm defined by an increase in red blood cell mass in the absence of a physiologic stimulus. The 2016 World Health Organization classification of myeloid neoplasms and acute leukemia has three major criteria and one minor criterion in the diagnosis of PV. Diagnosis requires either 3 major criteria or 2 major with 1 minor criterion (1).

Major Criteria for Polycythemia Vera

1. Increased hemoglobin level(>16.5 in men, >16 in women) or hematocrit(>49 in men, >48 in women)
2. Bone marrow biopsy showing hypercellularity for age with trilineage growth
3. JAK2 V617F or JAK2 exon 12 mutation

Minor Criterion for Polycythemia Vera

1. Serum erythropoietin level below the reference range of normal.

Due to the propensity of unusual thrombosis once a diagnosis is made therapy should be initiated without delay.  PV should be treated with phlebotomy followed by maintenance therapy with continued phlebotomy or hydroxyurea to maintain target hematocrit levels and with low dose aspirin (2,3). 

Hamayon Babary, MD and Muthena Maklad, MD

Department of Internal Medicine

University of Nevada School of Medicine: Las Vegas

Las Vegas, NV USA

References

1. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19;127(20):2391-405. [CrossRef] [PubMed]
2. Marchioli R, Finazzi F, Specchia G, et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013 Jan 3;368(1):22-33. [CrossRef] [PubMed]
3. Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, Barbui T; European Collaboration on Low-Dose Aspirin in Polycythemia Vera Investigators. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med. 2004 Jan 8;350(2):114-24. [CrossRef] [PubMed] 

Cite as: Babary H, Maklad M. Medical image of the week: portal vein thrombosis in a patient with polycythemia vera. Southwest J Pulm Crit Care. 2017;15(2):67-8. doi: https://doi.org/10.13175/swjpcc073-17 PDF