Sociedad Argentina de Hematología

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Revista Argentina de Hematología


Volumen:    21    # Number : 2

Publication Date :    Mayo - Agosto    Year:    2017


Portal hypertension in patient with myelofibrosis

Authors: Almeida L, Boedo L, Flores A, Mandrile A, Sgromo M

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.

Key words: Myelofibrosis, Sinister portal hypertension, JAK2 inhibitor, Ruxolitinib.

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