Hepatosplenomegaly in the course of the primary myelofibrosis in 49-year-old female patient – a case report
Keywords:
Hepatomegaly, splenomegaly, primary myelofibrosis, portal hypertensionAbstract
Primary myelofibrosis (primary myelofibrosis – PMF) belongs to group of rare, Philadelphia-negative myeloproliferative neoplasms. The essence of the disease is a bone marrow fibrosis, which is accompanied by the appearance of extramedullary hemopoiesis foci. Hematopoietic foci may be located within various organs, but by far the most occupied the liver and spleen. Creating alternative centers of hemopoiesis in these organs leads to increase their size with the development of portal hypertension and its complications. We present a rare case of hepatosplenomegaly in the course of primary myelofibrosis in 49-year-old female patient.
Downloads
References
Koch CA, Li CY, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin. Proc. 2003;78:1223.
Georgiades CS, Neyman EG, Francis IR, Sneider MB, Fishman EK. Typical and atypical presentations of extramedullary hemopoiesis. AJR Am J Roentgenol 2002; 179:1239.
Tefferi A. Primary myelofibrosis: 2012 update on diagnosis, risk stratification, and management. Am J Hematol. 2011;86:1017-26.
Tefferi A, Lasho TL, Jimma T, Finke CM, Gangat N, Vaidya R i wsp. One Thousand Patients With Primary Myelofibrosis: The Mayo Clinic Experience. Mayo Clin Proc. 2012;87:25-33.
Visani G, Finelli C, Castelli U, Petti MC, Ricci P i wsp. Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients. Br J Haematol. 1990;75:4-9.
Gangat N, Caramazza D, Vaidya R, George G, Begna K, Schwager S i wsp. DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol. 2011;29:392-7.
Vaidya R, Siragusa S, Huang J, Schwager SM, Hanson CA, Hussein K i wsp. Mature survival data for 176 patients younger than 60 years with primary myelofibrosis diagnosed between 1976 and 2005: evidence for survival gains in recent years. Mayo Clin Proc. 2009;84:1114-9.
Visani G, Finelli C, Castelli U, Petti MC, Ricci P, Vianelli N i wsp. Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients. Br J Haematol. 1990;75:4-9.
Bench AJ, Nacheva EP, Champion KM, Green AR. Molecular genetics and cytogenetics of myeloproliferative disorders. Bailliere’s Clin Haematol 1998;11:819-48.
Craig CE, Quaglia A, Dhillon AP. Extramedullary haematopoiesis in massive hepatic necrosis. Histopathology 2004;45:518-25.
Tefferi A, Mesa RA, Nagorney DM, Schroeder G, Silverstein MN. Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients. Blood 2000;95:2226-33.
Lataillade JJ, Pierre-Louis O, Hasselbalch HC, Uzan G, Jasmin C, Martyré MC i wsp. Does primary myelofibrosis involve a defective stem cell niche? From concept to evidence. Blood 2008 15;112:3026-35.
Seki S, Kitada T, Sakaguchi H, Iwai S, Kawada N, Hayashi Y i wsp. Expression of progenitor cell markers in livers with fulminant massive necrosis. Hepatol Res. 2003;25:149-57.
Prost S, LeDiscorde M, Haddad R, Gluckman JC, Canque B, Kirszenbaum M. Characterization of a novel hematopoietic marker expressed from early embryonic hematopoietic stem cells to adult mature lineages. Blood Cells Mol. Dis. 2002;29:236-48.
Roux D, Merlio JP, Quinton A, Lamouliatte H, Balabaud C, Bioulac-Sage P. Agnogenic myeloid metaplasia, portal hypertension, and sinusoidal abnormalities. Gastroenterology 1987;92:1067-72.
Tsao MS. Hepatic sinusoidal fibrosis in agnogenic myeloid metaplasia. Am J Clin Pathol 1989;91:302-05.
Wanless IR, Peterson P, Das A, Boitnott JK, Moore GW, Bernier V. Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: A clinicopathological study of 145 patients examined at autopsy. Hepatology 1990;12:1166-74.
Doki N, Irisawa H, Takada S, Sakura T, Miyawaki S. Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension due to idiopathic myelofibrosis. Intern Med. 2007;46:187-90.
Sacha T, Wącław J. Postępy w leczeniu samoistnego włóknienia szpiku. Hematologia 2012;3:1-7.
Czasopismo Publikacja
Pardanani A, Brown P, Neben-Wittich M, Tobin R, Tefferi A. Effective management of accelerated phase myelofibrosis with low-dose splenic radiotherapy Am J Hematol. 2010;85:715-6
Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M i wsp. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol. 2011 20;29:761-70.
Helbig G. Inhibitory JAK-czy jesteśmy świadkami przełomu w leczeniu włóknienia szpiku? Hematologia 2012;3:193–200.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2012 Medical Journal of the Rzeszow University and the National Medicines Institute, Warsaw

This work is licensed under a Creative Commons Attribution 4.0 International License.
Our open access policy is in accordance with the Budapest Open Access Initiative (BOAI) definition: this means that articles have free availability on the public Internet, permitting any users to read, download, copy, distribute, print, search, or link to the full texts of these articles, crawl them for indexing, pass them as data to software, or use them for any other lawful purpose, without financial, legal, or technical barriers other than those inseparable from having access to the Internet itself.
All articles are published with free open access under the CC-BY Creative Commons attribution license (the current version is CC-BY, version 4.0). If you submit your paper for publication by the Eur J Clin Exp Med, you agree to have the CC-BY license applied to your work. Under this Open Access license, you, as the author, agree that anyone may download and read the paper for free. In addition, the article may be reused and quoted provided that the original published version is cited. This facilitates freedom in re-use and also ensures that Eur J Clin Exp Med content can be mined without barriers for the research needs.




