Ruxolitinib - better prognostic impact in low-intermediate 1 risk score: evaluation of the 'rete ematologica pugliese' (REP) in primary and secondary myelofibrosis.
We evaluated ruxolitinib in 65 patients with myelofibrosis according to age, sex, time of diagnosis, grade of fibrosis, prognostic score risk, Janus kinase (JAK) status, primary or secondary myelofibrosis, previous treatment, and dosage. Outcome measures were response rate, time to response, duration of response, and event-free survival and survival. Kaplan and Meier curves show a significant difference in event-free survival according to the prognostic score, in favor of patients with low int1 (p = 0.0009). The Cox stepwise model confirmed the result, the int2 high-risk score being the most powerful negative independent parameter (0.001), followed by JAK (0.008); other parameters, such as diagnosis more than 5 years earlier, grade III-IV fibrosis, and ruxolitinib dose have a negligible impact. Time to response was shorter (p = 0.001) in primary myelofibrosis. In conclusion, ruxolitinib is effective, with a better outcome in patients with a low-int1 risk score. This may suggest considering an earlier administration in the disease course.
a Department of Hematology-Oncology , Complex Operative Unit of Hematology ASL , Taranto , Italy.
Patrizio
P
Mazza
b Department of Emergency and Organ Transplantation , Complex Operative Unit of Hematology , Bari , Italy.
Giorgina
G
Specchia
c Complex Operative Unit of Hematology - ASL , Lecce , Italy.
Nicola
N
Di Renzo
d Unit of Hematology and Bone Marrow Transplantation 'Casa Sollievo Della Sofferenza' S. Giovanni Rotondo , Foggia , Italy.
Nicola
N
Cascavilla
e Complex Operative Unit of Hematology - ASL BAT Andria , Italy.
Giuseppe
G
Tarantini
f Complex Operative Unit of Hematology , Foggia , Italy.
Silvana Franca
SF
Capalbo
a Department of Hematology-Oncology , Complex Operative Unit of Hematology ASL , Taranto , Italy.
Tiziana
T
Urbano
b Department of Emergency and Organ Transplantation , Complex Operative Unit of Hematology , Bari , Italy.
Francesco
F
Albano
c Complex Operative Unit of Hematology - ASL , Lecce , Italy.
Reddiconto
R
Giovannni
d Unit of Hematology and Bone Marrow Transplantation 'Casa Sollievo Della Sofferenza' S. Giovanni Rotondo , Foggia , Italy.
Antonietta Pia
AP
Falcone
e Complex Operative Unit of Hematology - ASL BAT Andria , Italy.
Maria Teresa
MT
Santeramo
f Complex Operative Unit of Hematology , Foggia , Italy.
Giuseppina
G
Spinosa
a Department of Hematology-Oncology , Complex Operative Unit of Hematology ASL , Taranto , Italy.
Salvatore
S
Pisconti
D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality*; D009570:Nitriles; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D011720:Pyrazoles / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D011743:Pyrimidines; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
D009570:Nitriles; D047428:Protein Kinase Inhibitors; D011720:Pyrazoles; D011743:Pyrimidines; C540383:ruxolitinib
Myelofibrosis; prognostic groups; response to ruxolitinib