Abstract
Portal hypertension occurs in approximately 10% of patients with myelofibrosis. Increased portal blood flow secondary to splenomegaly has been proposed to explain its development. In a 60-year-old woman with proven myelofibrosis of 10 years' duration and gross splenomegaly, portal hypertension developed with esophageal varices and ascites. There was no demonstrable obstruction to portal blood flow. Following splenectomy the ascites and esophageal varices disappeared. Despite the presence of splenic myeloid metaplasia, splenectomy did not impair the patient's hematologic status. Portal hypertension complicating myelofibrosis has a poor prognosis, so careful attention should be given to its detection. Splenectomy may be preferable to portal-systemic shunting in the management of this complication.
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- BOURONCLE B. A., DOAN C. A. Myelofibrosis. Clinical, hematologic and pathologic study of 110 patients. Am J Med Sci. 1962 Jun;243:697–715. doi: 10.1097/00000441-196206000-00001. [DOI] [PubMed] [Google Scholar]
- Blendis L. M., Banks D. C., Ramboer C., Williams R. Spleen blood flow and splanchnic haemodynamics in blood dyscrasia and other splenomegalies. Clin Sci. 1970 Jan;38(1):73–84. doi: 10.1042/cs0380073. [DOI] [PubMed] [Google Scholar]
- Gorshein D., Brauer M. J. Ascites in myeloid metaplasia due to ectopic peritoneal implantations. Cancer. 1969 Jun;23(6):1408–1412. doi: 10.1002/1097-0142(196906)23:6<1408::aid-cncr2820230623>3.0.co;2-f. [DOI] [PubMed] [Google Scholar]
- NAKAI G. S., CRADDOCK C. G., FIGUEROA W. G. Agnogenic myeloid metaplasia. A survey of twenty-nine cases and a review of the literature. Ann Intern Med. 1962 Sep;57:419–440. doi: 10.7326/0003-4819-57-3-419. [DOI] [PubMed] [Google Scholar]
- Rosenbaum D. L., Murphy G. W., Swisher S. N. Hemodynamic studies of the portal circulation in myeloid metaplasia. Am J Med. 1966 Sep;41(3):360–368. doi: 10.1016/0002-9343(66)90081-7. [DOI] [PubMed] [Google Scholar]
- SHALDON S., SHERLOCK S. Portal hypertension in the myeloproliferative syndrome and the reticuloses. Am J Med. 1962 May;32:758–764. doi: 10.1016/0002-9343(62)90165-1. [DOI] [PubMed] [Google Scholar]
- Schwartz S. I. Myeloproliferative disorders. Ann Surg. 1975 Oct;182(4):464–471. doi: 10.1097/00000658-197510000-00012. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Silverstein M. N., ReMine W. H. Sex, splenectomy, and myeloid metaplasia. JAMA. 1974 Jan 28;227(4):424–426. [PubMed] [Google Scholar]
- Silverstein M. N., Wollaeger E. E., Baggenstoss A. H. Gastrointestinal and abdominal manifestations of agnogenic myeloid metaplasia. Arch Intern Med. 1973 Apr;131(4):532–537. [PubMed] [Google Scholar]
- Sullivan A., Rheinlander H., Weintraub L. R. Esophageal varices in agnogenic myeloid metaplasia: disappearance after splenectomy. A case report. Gastroenterology. 1974 Mar;66(3):429–432. [PubMed] [Google Scholar]


