Abstract
We present the clinical case of a 54 year old female, previously diagnosed with polycythemia vera, consulting because of haematemesis and anemic syndrome. She also showed pallor, massive splenomegaly, leukocytosis, anemia and elevated LDH. An upper endoscopy reported gastric varicose veins, while abdominal doppler ultrasound and tomography reported signs of portal hypertension and ruled out splanchnic thrombosis. A bone marrow biopsy showed a myeloproliferative neoplasm in myelofibrotic stage. The patient was admitted at the hospital in several opportunities with varicose gastrointestinal bleeding, being local treatment unfeasible. Due to her lack of response with supportive treatment, TIPS and hydroxyurea, and assuming that portal hypertension as secondary to splenomegaly, a JAK2 inhibitor was started, ruxolitinb 15 mg bid, achieving clinical improvement, decrease of spleen size and stopping gastrointestinal bleeding episodes.
References
2. Koichi T, Hiroyuki M, Yukio Y, Shigeki Y. Mul-tiple esophageal variceal ruptures with massive ascities due to myelofibrosis-induced portal hy-pertension. World J Gastroenterol. 2012 Jul 28; 18(28): 3770-3774.
3. Abu-Hilal M, Tawaker J. Portal hypertension secondary to myelofibrosis with myeloid meta-plasia: A study of 13 cases. World J Gastroenter-ol. 2009 Jul 7; 15(25): 3128-3133.
4. Vannucchi A, Guglielmelli P. JAK2 Muta-tion-Related Disease and Thrombosis. Semin Thromb Hemost. 2013; 39:496–506.
5. Mughal T, Vaddi Kris, Sarlis N, Verstovek S. Myelofibrosis-associated complications: patho-genesis, clinical manifestations, and effects on outcomes. International Journal of General Medicine. 2014;7:89-101.
6. Glockenberg K, Weinberg E, Wan D. A sinis-ter cause of hematemesis. Gastroenterology. 2015;148(3):e5-e6.
7. Cervantes F. How I Treat Splenomegaly in my-elofibrosis. Blood Cancer Journal. 2011;1:e37.
8. Randhawa J et al. Splenomegaly in myelofibro-sis- new options for therapy and the therapeutic potential of Janus Kinase 2 inhibitors. Journal of Hemtatology & Oncology. 2012;5:43.
9. Koschmieder S, Koppelle A, Seifert H. Ru-xolitinib in myelofibrosis. N Engl J Med. 2012;366:21.
10. Stübig T, Alchalby H, Ditschkowski M et al. JAK inhibition with ruxolitinib as pretreatment for allogeneic stem cell transplantation in pri-mary or post-ET/PV myelofibrosis. Leukemia. 2014;28: 1736–1738.
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