Abstract

Philadelphia chromosome-negative myelopro-liferative neoplasms (MPN) are a heterogeneous group of hematological malignancies including essential thrombocythemia (ET), polycythemia (PV), and primary myelofibrosis (PMF). The MPN Symptom Assessment Form (SAF), or MPN 10, questionnaire is a validated tool for assessing the symptoms presented by patients with MPN and their evolution during treatment. To assess the impact of comorbidities and response to treatment on the symptomatology of patients with MPN assessed by the MPN SAF. This study, carried out in our hematology department, involved patients monitored and treated for MPN. We used the MPN SAF questionnaire, which includes 10 items rated from 0 to 10 in relation to the MPN symptom burden. Comorbidities were assessed using the Cumulative Illness Rating Scale (CIRS). Statistically, ANOVA and log rank tests were used to study the impact of comorbidities and response to treatment on MPN SAF scores. The tests are significant if P<0.05. Sixty-seven patients participated in this study, 31 women and 36 men, with a median age of 65 years [22-86]. Thirty-six (54%) patients had PV, 25 (37%) had ET, and 6 (9%) had PMF. Sixteen (24%) patients had splenomegaly, and half of these cases were bulky. The mean MPN SAF score was higher for patients with PMF (47) than for those with ET (26) or PV (21) (P=0.04). Forty-three (64%) patients had at least one associated disease, and 17 (25%) of these had a CIRS score ≥6. The MPN SAF scores were well correlated with the patients' comorbidity scores (P<0.001): patients with CIRS scores <6 had a mean MPN score of 48 versus 18 for patients with CIRS scores ≥6. Depending on the response to treatment and disease control, the MPN SAF scores were higher for patients refractory to treatment (mean: 35) than for those with complete or partial remission (17 and 22, respectively, P=0.02). Our study concludes that worsening of MPN symptoms is associated with comorbidities. It is very likely that associated diseases maintain a chronic inflammatory state that adds to that of chronic myeloid neoplasia, mainly when it is refractory to treatment.

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