Low-risk polycythemia vera treated with phlebotomies: clinical characteristics, hematologic control and complications in 453 patients from the Spanish Registry of Polycythemia Vera
Por:
Triguero, A, Pedraza, A, Perez-Encinas, M, Mata-Vazquez, MI, Velez, P, Fox, L, Gomez-Calafat, M, Garcia-Delgado, R, Gasior, M, Ferrer-Marin, F, Garcia-Gutierrez, V, Angona, A, Gomez-Casares, MT, Cuevas, B, Martinez, C, Perez, R, Raya, JM, Guerrero, L, Murillo, I, Bellosillo, B, Hernandez-Boluda, JC, Sanz, C, Alvarez-Larran, A
Publicada:
1 oct 2022
Ahead of Print:
1 ago 2022
Resumen:
Hematological control, incidence of complications, and need for cytoreduction were studied in 453 patients with low-risk polycythemia vera (PV) treated with phlebotomies alone. Median hematocrit value decreased from 54% at diagnosis to 45% at 12 months, and adequate hematocrit control over time (<45%) was observed in 36%, 44%, and 32% of the patients at 6, 12, and 24 months, respectively. More than 5 phlebotomies per year in the maintenance phase were required in 19% of patients. Worsening thrombocytosis, age > 60 years, and microvascular symptoms constituted the main indications for starting cytoreduction. Median duration without initiating cytoreduction was significantly longer in patients younger than 50 years (< 0.0001). The incidence rate of thrombosis under phlebotomies alone was 0.8% per year and the estimated probability of thrombosis at 10 years was 8.5%. The probability of arterial thrombosis was significantly higher in patients with arterial hypertension whereas there was a trend to higher risk of venous thrombosis in cases with high JAK2V617F allele burden. Rates of major bleeding and second primary neoplasm were low. With a median follow-up of 9 years, survival probability at 10 years was 97%, whereas the probability of myelofibrosis at 10 and 20 years was 7% and 20%, respectively. Progression to acute myeloid leukemia was documented in 3 cases (1%). Current management of low-risk PV patients is associated with low rate of thrombosis and long survival. New treatment strategies are needed for improving hematological control and, in the long term, reducing progression to myelofibrosis.
Filiaciones:
Triguero, A:
Hosp Clin Barcelona, Barcelona, Spain
Pedraza, A:
Hosp Clin Barcelona, Barcelona, Spain
Perez-Encinas, M:
Complejo Hosp Univ Santiago, Santiago De Compostela, Spain
Mata-Vazquez, MI:
Hosp Costa del Sol, Marbella, Spain
Velez, P:
Hosp Mar, Barcelona, Spain
Fox, L:
Hosp Univ Vall dHebron, Vall dHebron Inst Oncol VHIO, Hematol Expt, Barcelona, Spain
Gomez-Calafat, M:
Hosp Clin Univ Valencia, Valencia, Spain
Garcia-Delgado, R:
Hosp Univ Virgen Victoria, Malaga, Spain
Gasior, M:
Hosp Univ La Paz, Madrid, Spain
Ferrer-Marin, F:
Hosp Univ Morales Meseguer, CIBERER UCAM, Murcia, Spain
Garcia-Gutierrez, V:
Hosp Univ Ramon Y Cajal IRYCIS, Madrid, Spain
Angona, A:
ICO Girona Hosp Josep Trueta, Girona, Spain
Gomez-Casares, MT:
Hosp Univ Gran Canaria Dr Negrin, Las Palmas Gran Canaria, Spain
Cuevas, B:
Hosp Univ Burgos, Burgos, Spain
Martinez, C:
Hosp Santa Creu & Sant Pau, Barcelona, Spain
Perez, R:
Hosp Univ Clin Virgen Arrixaca, Murcia, Spain
Raya, JM:
Hosp Univ Canarias, Tenerife, Spain
Guerrero, L:
Hosp Rio Carrion, Palencia, Spain
Murillo, I:
Hosp Gen San Jorge, Huesca, Spain
Bellosillo, B:
Hosp Mar, Barcelona, Spain
Hernandez-Boluda, JC:
Hosp Clin Univ Valencia, Valencia, Spain
Sanz, C:
Hosp Clin Barcelona, Barcelona, Spain
Alvarez-Larran, A:
Hosp Clin Barcelona, Barcelona, Spain
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