TY - JOUR
T1 - 20+ Years and alive with primary myelofibrosis
T2 - Phenotypic signature of very long-lived patients
AU - Penna, Domenico
AU - Lasho, Terra L.
AU - Finke, Christy M.
AU - Vallapureddy, Rangit R.
AU - Hanson, Curtis A.
AU - Ketterling, Rhett P.
AU - Pardanani, Animesh
AU - Gangat, Naseema
AU - Tefferi, Ayalew
N1 - Publisher Copyright:
© 2018 Wiley Periodicals, Inc.
PY - 2019/3
Y1 - 2019/3
N2 - In the last decade, several prognostic models for primary myelofibrosis (PMF) have been introduced and shown to be effective in predicting overall survival. The main objective for this study was to identify clinical and genetic markers of very long (20+ years) survival in PMF. A total of 1282 patients with PMF were considered (median age 65 years, range 19-92; 63% males); 26 (2%) patients (median age 51 years, range 28-71; 38% males) survived their disease for at least 20 years (long-lived patients) and 626 (49%) patients (median age 68 years, range 27-92; 66% males) died within 5 years of their diagnosis (short-lived patients). Multivariable logistic regression analysis identified 7 variables that were associated with survival beyond 20 years: age ≤ 70 years (P =.002); female sex (P =.03); hemoglobin level ≥ 10 g/dL for women and ≥ 11 g/dL for men (P =.03), leukocyte count ≤25 × 10 9 /L (P =.009), platelet count ≥100 × 10 9 /L (P =.002), circulating blasts <2% (P =.03) and absence of constitutional symptoms (P =.04). Five-year mortality was independently predicted by high-molecular risk mutations (P <.001); unfavorable or very high risk karyotype (P <.001); absence of type 1/like CALR mutation (P <.001); age > 70 years (P <.001); constitutional symptoms (P <.001); hemoglobin level < 10 g/dL for women and < 11 g/dL for men (P <.001); leukocyte count >25 × 10 9 /L (P =.004); and circulating blasts ≥2% (P =.001). This study suggests that genetic risk factors in PMF are associated with early mortality while survival beyond 20 years could be predicted by easily accessible clinical variables, including age, sex, blood counts, and symptoms.
AB - In the last decade, several prognostic models for primary myelofibrosis (PMF) have been introduced and shown to be effective in predicting overall survival. The main objective for this study was to identify clinical and genetic markers of very long (20+ years) survival in PMF. A total of 1282 patients with PMF were considered (median age 65 years, range 19-92; 63% males); 26 (2%) patients (median age 51 years, range 28-71; 38% males) survived their disease for at least 20 years (long-lived patients) and 626 (49%) patients (median age 68 years, range 27-92; 66% males) died within 5 years of their diagnosis (short-lived patients). Multivariable logistic regression analysis identified 7 variables that were associated with survival beyond 20 years: age ≤ 70 years (P =.002); female sex (P =.03); hemoglobin level ≥ 10 g/dL for women and ≥ 11 g/dL for men (P =.03), leukocyte count ≤25 × 10 9 /L (P =.009), platelet count ≥100 × 10 9 /L (P =.002), circulating blasts <2% (P =.03) and absence of constitutional symptoms (P =.04). Five-year mortality was independently predicted by high-molecular risk mutations (P <.001); unfavorable or very high risk karyotype (P <.001); absence of type 1/like CALR mutation (P <.001); age > 70 years (P <.001); constitutional symptoms (P <.001); hemoglobin level < 10 g/dL for women and < 11 g/dL for men (P <.001); leukocyte count >25 × 10 9 /L (P =.004); and circulating blasts ≥2% (P =.001). This study suggests that genetic risk factors in PMF are associated with early mortality while survival beyond 20 years could be predicted by easily accessible clinical variables, including age, sex, blood counts, and symptoms.
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U2 - 10.1002/ajh.25351
DO - 10.1002/ajh.25351
M3 - Article
C2 - 30516867
AN - SCOPUS:85057728620
SN - 0361-8609
VL - 94
SP - 286
EP - 290
JO - American journal of hematology
JF - American journal of hematology
IS - 3
ER -