Symptom Assessment in Patients with BCR-ABL-Negative Myeloproliferative Neoplasms at an Academic Medical Institution

Background: Patients with BCR-ABL-negative myeloproliferative neoplasms (MPNs) have high symptom burdens that negatively impact quality of life, including risk for developing chronic pain and psychosocial complications. The NCCN guidelines recommended the use of the MPN Symptom Assessment Form Total Symptom Score (MPN-SAF TSS) starting in 2017 to assess symptom burden. Our primary aim was to determine if the MPN-SAF TSS has been incorporated into patient care, and if not, how well BCR-ABL-negative MPN symptoms are captured by review of systems (ROS). Our secondary aim was to evaluate the prevalence of anxiety, depression, chronic pain, and opiate use in our patient population. Methods: We performed a single-center, cross-sectional study of all active BCR-ABL-negative MPN patients in the UCSF Hematology Clinic between January 2017 and March 2019. We reviewed all hematology visits for completion of the MPN-SAF TSS, and the most recent visit for the number symptoms from the MPN-SAF TSS explicitly captured in ROS or problem list and relevant medications. Descriptive statistics were used to summarize the data. Patients whose disease transformed into acute leukemia or were post-allogeneic stem cell transplantation were excluded. Results: Of 299 patients with BCR-ABL-negative MPN diagnoses, the median age was 66 (range 20-98) with an equal number of males (n=150) and females (n=149). Essential thrombocythemia (ET) was the most common diagnosis (n=109; 37%), followed by polycythemia vera (PV) (n=90; 30%), primary myelofibrosis (MF) (n=49; 16%), post-PV or post-ET MF (n=29; 10%), and MPN-Unclassifiable or overlap (n=22; 7%). Most were JAK2 V617F positive (n=213; 71%) and high-risk (n=148; 49.5%) by clinical criteria, IPSET-thrombosis, and DIPSS-plus. Nearly all were on active treatment (91%), with aspirin (n=205; 69%), hydroxyurea (n=130; 43.5%), phlebotomy (n=61; 20%), and ruxolitinib (n=37; 12%) being the most frequent treatments. Significant disease-related vascular complications were documented in 20.7% of patients. The 299 patients were evaluated by 22 hematology providers. The MPN-SAF TSS was formally documented in 1 patient (0.3%). Of the 10 symptoms in the MPN-SAF TSS, the median number documented as positive or negative on ROS was 3 (range 0-8), with 0 or 1 symptoms documented in 82 patients (27.4%). The mean number of positive symptoms was 0.7 (range 0-4) with at least 1 positive symptom reported by 44.7%. The most frequently charted symptoms were fever (