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Vol. 42. Issue S2.
Pages 512 (November 2020)
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Vol. 42. Issue S2.
Pages 512 (November 2020)
862
Open Access
POLYCYTHEMIA VERA: AN EPIDEMIOLOGIC ANALYSIS AND PATIENT FOLLOW UP
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G.C.B. Valente, R.O. Coelho, L.F.M. Reynaldo, L.F. Silva
Pontifícia Universidade Católica de Campinas (PUC-Campinas), Campinas, SP, Brazil
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Objectives: The objectives were to identify the demographic characteristics and the method of treatment of the patients diagnosed with Polycythemia Vera (PV, CID-10 D45.0) in theservice, and to help the institution on the creation of its own protocol for diagnosis and treatment of such patients. Material and Methods: This retrospective and observational study was made with an analysis of data from 38 patients diagnosed with PV. The data range from 1998 to 2019 and was collected using a protocol established by the researchers. Results: The sample features 60,5% of male and medium age of 57 years, 17 of those were classified as high risk patients. Observing the cardiovascular risk factors, the most prevalentwas Arterial Hypertension (55,3%). The most related clinical finding was plethora (68,4%) followed by splenomegaly (42,1%). Five patients had thromboembolic events before the treatment and four had after it. As a treatment, almost all patients used hydroxyurea, with some using phlebotomy as prophylactic and/or maintenance option. Three of the patients evolved to myelofibrosis. Only six patients died during years studied and the survival ratewas of 19,8 years after diagnosis. Discussion: Polycythemia Rubra Vera is the most common myeloproliferative disease, occurring in 1,9/100.000 inhabitants (USA). The disease has a discrete predominance over men, grows in incidence after the 6th decade and occurs earlier in women. The disease manifests with symptoms occurring from blood hyperviscosity orvasomotor factors, such as fatigue, headache, visual disturbance and pruritus. Splenomegaly is the most common sign. Clinical findings can persist with laboratory control of the disease and can aggravate with its evolution. As such, clinical parameters are unreliable factors for patient's control. Given the unspecific clinical findings, the diagnosis is established following a set of criteria anchored in laboratory findings and guided by WHO's guideline of 2016. Risk stratification is fundamental to establish the correct treatment, in an attempt of thrombosis prevention. The patient is considered as “high risk” if its age is over 60 years or it has a history of previous thrombosis. Modern protocols for “low risk” patients recommend phlebotomy until hematocrittarget level is reached and daily low dose aspirin. For “high risk” patients the recommendation is hydroxyurea and to evaluate the response, preceded by phlebotomy until target hematocrit levels. Antiplatelets agents, anticoagulants, phlebotomy and other drugs can be used in special cases. The diseases natural history is not well defined, mainly due to the established treatments that change its progression. Yet, life expectancy is lower compared to the overall population. Conclusion: The patient's array was epidemiologically consistent with the literature. However, symptomatology data was shown to be lower than reported in other studies. Phlebotomy was used in agreement with literature (when the hematocrit was over 45%). However, the usage of hydroxyurea in lower-risk patients disagrees with international treatment protocols. This may have been due to the patients socioeconomic conditions which may make it difficult for the patient to receive phlebotomy only treatment. Despite this divergency in treatment, the study showed a lower thrombosis rate than what has been reported in the literature.

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Hematology, Transfusion and Cell Therapy
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