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

Medical Image of the week: ECHO findings of Apical Ballooning Syndrome

  Figure 1. Panel A: Four chamber view of the heart at the end of diastole with a dilated left ventricle. Panel B: Same four chamber view of the heart at the end of systole with a dilation and akinesis of the apical portion (arrow) of the heart consistent with apical ballooning/stress cardiomyopathy

A 79 year old man with a history of lung cancer. bladder cancer, chronic obstructive pulmonary disease, and coronary artery disease with two previous myocardial infarctions, presented to the emergency department with respiratory failure secondary to pulmonary edema. Further evaluation was significant for non-ST segment elevation myocardial infarction. Cardiac catheterization was remarkable for a focal, eccentric 95% stenosis of the proximal to mid left anterior descending artery that failed stenting due to extensive calcifications. An echocardiogram (ECHO, Figure 1) revealed an ejection fraction of 47% with akinesis of the mid to distal anterior, lateral, inferior, septal and apical segments consistent with takotsubo cardiomyopathy.

Takotsubo cardiomyopathy, aka apical ballooning syndrome or stress induced cardiomyopathy, is a subtype of heart failure typically defined by proposed criteria from the Mayo Clinic that includes: 1. transient hypokinesis, akinesis, or dyskinesis in the left ventricular mid segments with or without apical involvement frequently occurring, but not always, in context to a stressful trigger; 2. the absence of angiographic evidence of obstructive coronary disease or plaque rupture; 3. new ECG abnormalities (ST-segment elevation and/or T-wave inversion) or modest elevation in cardiac troponin; and 4. the absence of pheochromocytoma and myocarditis (1). It predominantly occurs in post-menopausal women in relation to unexpected emotional or physical stress. Characteristic ECHO findings demonstrate a symmetrical regional wall motion abnormalities extending equally into the apical inferior and lateral wall with overall global hypokinesia (Figure 1) (2).

Sachin Kalarn MSIV; Sophie Galson MD; Kristina Skinner DO; Ryan Nahapetian MD, MPH

University of Arizona

Tucson, AZ

References

1. Akashi YJ, Goldstein DS, Barbaro G, Ueyama T. Takotsubo cardiomyopathy: a new form of acute, reversible heart failure. Circulation. 2008;118(25):2754-62. [CrossRef] [PubMed]
2. Chockalingam A, Xie GY, Dellsperger KC. Echocardiography in stress cardiomyopathy and acute LVOT obstruction. Int J Cardiovasc Imaging. 2010;26(5):527-35. [CrossRef] [PubMed]

Reference as: Kalarn S, Galson S, Skinner K, Nahapetian R. Medical image of the week: ECHO findings of aprical ballooning syndrome. Southwest J Pulm Crit Care. 2015;10(4):150-1. doi: http://dx.doi.org/10.13175/swjpcc024-15 PDF