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Esophageal Variceal Hemorrhage Secondary to Post-Polycythemic Myelofibrosis

Esophageal Variceal Hemorrhage Secondary to Post-Polycythemic Myelofibrosis Downloaded from https://academic.oup.com/jcag/article-abstract/1/3/95/4999627 by guest on 17 October 2018 Journal of the Canadian Association of Gastroenterology, 2018, 1(3), 95–96 doi: 10.1093/jcag/gwy016 Image of the Month Advance Access publication 18 May 2018 Image of the Month Esophageal Variceal Hemorrhage Secondary to Post-Polycythemic Myelofibrosis Figure  1. Upper endoscopy demonstrating medium esophageal varices with high-risk stig- mata. A  fibrin clot was present at the distal aspect of one column (not shown). He also had nonbleeding gastroesophageal varices (GOV1 and GOV2) (not shown). Figure  2. Axial CT sections demonstrating massive splenomegaly with a severely dilated A 71-year-old male presented to the emergency department splenic vein. with hematochezia and a hemoglobin drop from 90 to 70 g/L. His medical history was significant for polycythemia rubra vera that transformed to myelofibrosis (MF), but there was myeloid cells or increased portal flows secondary to spleno - no known liver disease. On examination, he was hemody- megaly (3, 4). namically stable with marked splenomegaly, but he had no Small studies have shown PH develops in approximately other signs of portal hypertension (PH). Bloodwork demon- 11% of MF individuals; however, the National Comprehensive strated normal liver enzymes and function. Upper endoscopy Cancer Network provides no guidance on screening for portal showed varices (Figure  1). CT venogram revealed patent hypertension (2, 4). Traditional strategies to treat complications portal and hepatic veins with no thrombus. Massive spleno- of cirrhotic PH have been utilized for MF, including EVL, NSBB megaly (>30  cm) was noted. A  severely dilated splenic vein and transjugular intrahepatic portosystemic shunting (1, 2). (2.5 cm) communicated with an enlarged portal vein (1.8 cm) Given the frequency of PH in MF and potential catastrophic (Figure 2). Endoscopic variceal ligation (EVL) was performed complications, further study should explore potential merits of and nonselective beta-blocker (NSBB) therapy was initiated endoscopic screening. before discharge. As a complication of MF, PH can occur without develop- ACKNOWLEDGEMENTS ment of portal vein thrombus (1). PH has rarely been docu- mented in post-polycythemic MF (2). The mechanism of PH DM described the case. DM and TK conducted the literature review in absence of thrombosis is proposed to be extramedullary and wrote the manuscript. TK conceived the study. IE supervised the hematopoiesis in the liver and infiltration of sinusoids with project. © The Author(s) 2018. Published by Oxford University Press on behalf of the Canadian Association of Gastroenterology. 95 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jcag/article-abstract/1/3/95/4999627 by guest on 17 October 2018 96 Journal of the Canadian Association of Gastroenterology, 2018, Vol. 1, No. 3 Douglas Motomura, MD , Tasha Kulai, MD, References 2 3 FRCPC , and Ian Epstein, MD, FRCPC 1. Alvarez-Larrán A, Abraldes JG, Cervantes F, et  al. Portal hyper- tension secondary to myelofibrosis: A  study of three cases. Am J Division of General Internal Medicine, Dalhousie University, Gastroenterol 2005;100:2355–8. Halifax, Nova Scotia, Canada; Division of Digestive Care and 2. Abu-Hilal M, Tawaker J. Portal hypertension secondary to myelo- Endoscopy, Dalhousie University Halifax, Nova Scotia, Canada; fibrosis with myeloid metaplasia: A  study of 13 cases. World J Division of Digestive Care and Endoscopy, Dalhousie University, Gastroenterol 2009;15:3128–33. Halifax, Nova Scotia, Canada 3. Roux D, Merlio JP, Quinton A, et  al. Agnogenic myeloid meta- plasia, portal hypertension, and sinusoidal abnormalities. Correspondence: Douglas Motomura, MD, Department of Gastroenterology 1987;92:1067–72. Medicine, Dalhousie University & Nova Scotia Health Authority, 4. National Comprehensive Cancer Network. Myeloproliferative neo- QEII Health Sciences Centre, VG Site, Suite 442 Bethune plasms (Version 2.2018). <hps://w tt ww.nccn.org/store/login/login. Building , 1276 South Park Street, Halifax, NS B3H 2Y9. E-mail aspx?ReturnURL=https%3a%2f%2fwww.nccn.org%2fprofession- dmotomura@dal.ca. als%2fphysician_gls%2fpdf%2fmpn.pdf>. (Accessed March 7, 2018). http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of the Canadian Association of Gastroenterology Oxford University Press

Esophageal Variceal Hemorrhage Secondary to Post-Polycythemic Myelofibrosis

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Oxford University Press
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© The Author(s) 2018. Published by Oxford University Press on behalf of the Canadian Association of Gastroenterology.
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2515-2084
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2515-2092
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10.1093/jcag/gwy016
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Abstract

Downloaded from https://academic.oup.com/jcag/article-abstract/1/3/95/4999627 by guest on 17 October 2018 Journal of the Canadian Association of Gastroenterology, 2018, 1(3), 95–96 doi: 10.1093/jcag/gwy016 Image of the Month Advance Access publication 18 May 2018 Image of the Month Esophageal Variceal Hemorrhage Secondary to Post-Polycythemic Myelofibrosis Figure  1. Upper endoscopy demonstrating medium esophageal varices with high-risk stig- mata. A  fibrin clot was present at the distal aspect of one column (not shown). He also had nonbleeding gastroesophageal varices (GOV1 and GOV2) (not shown). Figure  2. Axial CT sections demonstrating massive splenomegaly with a severely dilated A 71-year-old male presented to the emergency department splenic vein. with hematochezia and a hemoglobin drop from 90 to 70 g/L. His medical history was significant for polycythemia rubra vera that transformed to myelofibrosis (MF), but there was myeloid cells or increased portal flows secondary to spleno - no known liver disease. On examination, he was hemody- megaly (3, 4). namically stable with marked splenomegaly, but he had no Small studies have shown PH develops in approximately other signs of portal hypertension (PH). Bloodwork demon- 11% of MF individuals; however, the National Comprehensive strated normal liver enzymes and function. Upper endoscopy Cancer Network provides no guidance on screening for portal showed varices (Figure  1). CT venogram revealed patent hypertension (2, 4). Traditional strategies to treat complications portal and hepatic veins with no thrombus. Massive spleno- of cirrhotic PH have been utilized for MF, including EVL, NSBB megaly (>30  cm) was noted. A  severely dilated splenic vein and transjugular intrahepatic portosystemic shunting (1, 2). (2.5 cm) communicated with an enlarged portal vein (1.8 cm) Given the frequency of PH in MF and potential catastrophic (Figure 2). Endoscopic variceal ligation (EVL) was performed complications, further study should explore potential merits of and nonselective beta-blocker (NSBB) therapy was initiated endoscopic screening. before discharge. As a complication of MF, PH can occur without develop- ACKNOWLEDGEMENTS ment of portal vein thrombus (1). PH has rarely been docu- mented in post-polycythemic MF (2). The mechanism of PH DM described the case. DM and TK conducted the literature review in absence of thrombosis is proposed to be extramedullary and wrote the manuscript. TK conceived the study. IE supervised the hematopoiesis in the liver and infiltration of sinusoids with project. © The Author(s) 2018. Published by Oxford University Press on behalf of the Canadian Association of Gastroenterology. 95 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jcag/article-abstract/1/3/95/4999627 by guest on 17 October 2018 96 Journal of the Canadian Association of Gastroenterology, 2018, Vol. 1, No. 3 Douglas Motomura, MD , Tasha Kulai, MD, References 2 3 FRCPC , and Ian Epstein, MD, FRCPC 1. Alvarez-Larrán A, Abraldes JG, Cervantes F, et  al. Portal hyper- tension secondary to myelofibrosis: A  study of three cases. Am J Division of General Internal Medicine, Dalhousie University, Gastroenterol 2005;100:2355–8. Halifax, Nova Scotia, Canada; Division of Digestive Care and 2. Abu-Hilal M, Tawaker J. Portal hypertension secondary to myelo- Endoscopy, Dalhousie University Halifax, Nova Scotia, Canada; fibrosis with myeloid metaplasia: A  study of 13 cases. World J Division of Digestive Care and Endoscopy, Dalhousie University, Gastroenterol 2009;15:3128–33. Halifax, Nova Scotia, Canada 3. Roux D, Merlio JP, Quinton A, et  al. Agnogenic myeloid meta- plasia, portal hypertension, and sinusoidal abnormalities. Correspondence: Douglas Motomura, MD, Department of Gastroenterology 1987;92:1067–72. Medicine, Dalhousie University & Nova Scotia Health Authority, 4. National Comprehensive Cancer Network. Myeloproliferative neo- QEII Health Sciences Centre, VG Site, Suite 442 Bethune plasms (Version 2.2018). <hps://w tt ww.nccn.org/store/login/login. Building , 1276 South Park Street, Halifax, NS B3H 2Y9. E-mail aspx?ReturnURL=https%3a%2f%2fwww.nccn.org%2fprofession- dmotomura@dal.ca. als%2fphysician_gls%2fpdf%2fmpn.pdf>. (Accessed March 7, 2018).

Journal

Journal of the Canadian Association of Gastroenterology – Oxford University Press

Published: Sep 12, 2018

References

  • Portal hypertension secondary to myelofibrosis: A study of three cases
  • Portal hypertension secondary to myelofibrosis with myeloid metaplasia: A study of 13 cases
  • Agnogenic myeloid metaplasia, portal hypertension, and sinusoidal abnormalities