Palabras clave: calidad de vida, mielofibrosis, neoplasias mieloproliferativas, esplenomegalia, ruxolitinib
ABSTRACT Background: Myeloproliferative neoplasms can shorten the life of patients and affect severely their quality of life as a result of the severe constitutional symptoms mediated by cytokines resulting from massive splenomegaly characteristic of these diseases. The objective measurement of symptoms of myelofibrosis is essential for the presence of general constitutional symptoms and for assessing the outcome of treatment with JAK-2 inhibitors. Objective: To assess the impact on quality of life in patients with myeloproliferative neoplasms treated with the inhibitor of JAK-1 and JAK-2 ruxolitinib. Material and method: There was applied the questionnaire Myelofibrosis Symptom Assessment Form to 16 patients included in the Compassionate Use of Ruxolitinib Program: eight men and eight women were included with a median age of 63 years; four patients had primary myelofibrosis, three had postpolycythemia vera myelofibrosis and nine essential postthrombocythemia myelofibrosis. The patients started with a dose of 20 mg ruxolitinib orally every 12 hours. Results: The questionnaire was administered to all patients at each visit. By comparing the differences of each parameter measured between the start and at 3, 6 and 12 months were statistically significant for all variables (p < 0.001, 95%). The most significant changes were observed when comparing data at baseline and follow-up to the maximum time; however, were obvious from the first quarter of observation on fatigue and symptoms of splenomegaly in most general symptoms, which disappeared completely after three months of treatment. The variables with lower proportional change recorded were general activity, mood, walking tolerance and everyday work. Conclusions: The treatment of myeloproliferative neoplasms with ruxolitinib represents a major advance in the management of myeloproliferative diseases and achieved excellent improvement in overall constitutional symptom control, splenomegaly and the general perception of the quality of life of these patients. The objective measurement of symptoms of myelofibrosis is essential for assessing the outcome of treatment with inhibitors JAK-2. Key words: myelofibrosis, myeloproliferative neoplasms, splenomegaly, quality of life, ruxolitinib.
Keywords: quality of life, myelofibrosis, myeloproliferative neoplasms, splenomegaly, ruxolitinib