Sociedad Argentina de Hematología

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ISSN 2250-8309

 

 

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Revista Argentina de Hematología

Abstract

Volumen:    21    # Number : 2

Publication Date :    MAYO - AGOSTO    Year:    2017

   ATENEO

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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SOCIEDAD ARGENTINA DE HEMATOLOGÍA
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