More than half of patients with indolent systemic mastocytosis (SM) reported mild or worse quality of life effects from the disease, including at least mild impairment, according to results from a study published recently in JEADV Clinical Practice.
This study of 52 adult patients found that women and those with recent symptom onset experienced greater challenges, particularly in social and psychological aspects of their lives. The findings underscored the need for personalized care strategies to address the diverse experiences of individuals living with indolent SM.
What is SM?
Systemic mastocytosis (SM) is a rare hematological disease characterized by mast cells that are overactive and accumulate in different parts of the body such as the bone marrow, liver, spleen, gastrointestinal tract and lymph nodes.
The “stark gender difference that emerged from our data stands a novel finding; notably, this difference persisted also when controlling for other possible confounding factors, such as duration of disease and the presence of cutaneous symptoms,” the study authors said.
Read more about SM signs and symptoms
Among the 52 participants, most reported skin symptoms (86.5%) or visible lesions (88.5%), which were closely tied to diminished quality of life. Cutaneous symptoms particularly influenced scores on a dermatology life quality index and the mastocytosis quality of life questionnaire.
Interestingly, while skin involvement affected daily life (as shown in dermatology life quality index scores), the visibility of lesions had less effect on mastocytosis quality of life questionnaire scores, suggesting nuanced differences in how physical and emotional aspects were perceived.
Sex-based differences were notable, with female patients reporting higher impairment of quality of life in both questionnaires. The disparity may reflect social or cultural pressures or potentially differing psychological responses to the disease.
In addition, patients diagnosed within the past five years experienced more severe effects on quality of life across all mastocytosis quality of life questionnaire subdomains except for skin-specific issues. These patients may have faced challenges adapting to a new diagnosis or understanding their condition.
Skin symptoms and lesion location played a critical role in patients’ experiences. Lesions on visible areas, such as the hands, were associated with significantly higher dermatology life quality index scores, highlighting the stigma or self-consciousness they may provoke. Although the extent of cutaneous involvement did not correlate directly with quality of life scores, symptoms such as itching or flushing consistently worsened overall quality of life measures.
A moderately strong correlation was found between dermatology life quality index and mastocytosis quality of life questionnaire scores, particularly in domains related to skin and social life. This suggested that skin-related issues substantially affected both physical comfort and social interactions.
The study also highlighted its limitations, including a design at a single center and a small sample size, which may not fully capture the diversity of experiences with indolent SM.
The results emphasized the importance of comprehensive care for patients with indolent SM, addressing both physical symptoms and emotional well-being. Future research could refine tools for assessing quality of life and explore tailored interventions to alleviate the disease’s multifaceted burden. For patients, understanding the effects may encourage open conversations with health care providers about strategies to improve their quality of life.
