Factors Associated with Quality of Life Impairments in Patients with Myeloproliferative Neoplasms: Looking Beyond Symptom Burden
Authors: How Chi-Joan , Coffey Bridget , Dieli-Conwright Christina M. , Hobbs Gabriela S. , Amonoo Hermioni L , El-Jawahri Areej , Newcomb Richard 
Published: December 2024

INTRODUCTION
Patients with myeloproliferative neoplasms (MPNs) including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF) experience inflammatory symptoms, thrombotic complications, and increased risk of progression to more aggressive leukemias that result in quality of life (QOL) impairments. However, the relationship between symptoms, psychological distress, illness perception, coping, and perception of prognosis and QOL are unknown. Understanding these factors at the time of MPN diagnosis and how these factors interact with symptomology is important for effective patient education on living with MPN and informs development of novel supportive care interventions.

OBJECTIVES
We sought to describe psychological distress, illness perception, coping, and perception of prognosis in newly diagnosed patients with MPN and evaluate their associations with QOL when controlling for symptom burden.

METHODS
We conducted cross-sectional analyses of data from 34 adults newly diagnosed with MPN at a single academic center. We enrolled consecutive patients who were diagnosed &lt;3 months prior to study enrollment. We obtained patient demographics, clinical characteristics, and patient reported outcomes to assess MPN symptom burden (MPN Symptom Assessment Form Total Symptom Score [MPN SAF TSS]), psychological distress (Hospital Anxiety and Depression Scale [HADS-A/D]), illness perception (Brief Illness Perception Questionnaire [BIPQ]), coping (Brief COPE), and QOL (Functional Assessment of Cancer Therapy [FACT-G]). Clinically significant symptoms and psychological distress were defined as MPN SAF TSS scores &gt;10 and HADS-A or HADS-D score &gt;7. Moderately and severely threatening perceptions of illness were defined as BIPQ scores 42-49 and &gt;50. We used the median split method to define higher and lower use of approach-oriented (positive re-framing, problem solving, seeking emotional support) and avoidant-oriented (denial, avoidance, and disengagement) coping strategies. We used unadjusted linear and logistic regression models to identify factors that are associated with QOL. Multivariate linear regression models controlling for symptom burden were used to independently examine associations between psychological distress, illness perception, or coping with QOL.

RESULTS
We enrolled 34 patients with MPN (13 ET; 12 PV; 8 MF; 1 MPN-U; median age 68.1; range 33.31-82.06; 52.9% female). Mean MPN SAF TSS score was 14.88 with no significant differences among MPN subtypes (p=0.84). Clinically significant anxiety or depression was seen in 17.65% and 11.76% of MPN patients, respectively. 27.27% of MPN patients had moderately or severely threatening perceptions of their illness. 41.2% of MPN patients highly used approach-oriented coping strategies at the time of diagnosis, and 35.3% of MPN patients highly used avoidant-oriented coping mechanisms. On univariate analysis, MPN SAF TSS (B=-19.07; p&lt;0.001), BIPQ (B=-33.38; p&lt;0.001 for scores &gt;50; B=-13.813; p=0.04 for scores 42-49), HADS-A (B=-23.87; p=0.001) and D scores (B=-37.547; p&lt;0.001), and avoidant-oriented coping strategies (B=-17.35; p=0.004) were associated with lower FACT-G scores, corresponding to more impaired QOL. BIPQ (B=-13.821; p&lt;0.001), HADS-A (B=-20.47; p&lt;0.001) and HADS-D (B=-30.86; p&lt;0.001) scores, and high use of avoidant-oriented coping strategies (B=-11.73; p=0.04) remained significantly associated with lower FACT-G scores even when controlling for symptom burden.

CONCLUSIONS
A significant proportion of newly diagnosed patients with MPN experience psychological distress, report moderately to severely threatening illness perception, and highly use avoidant coping strategies. Symptom burden remains associated with QOL decrements. However, our analysis also suggests that QOL is impacted by other aspects of illness which are independent of symptoms, including anxiety, depression, perception of disease severity, and maladaptive coping. Supportive care interventions to address patients' disease understanding and expectations, coping needs, and psychological distress are needed in this vulnerable population.