A new study has characterized the symptomatology of patients with advanced systemic mastocytosis (SM) as reported by oncologists at the first patient visit. The study, published in Clinical Lymphoma, Myeloma & Leukemia, found a wide range of symptoms, comorbid conditions, and diagnostic strategies for these patients.

“Although driven by KIT D816V in 95% of cases, the heterogeneous symptom presentation of [advanced SM] patients may contribute to a lengthy diagnostic journey,” the authors wrote. They are presenting their results at the Tenth Annual Meeting of the Society of Hematologic Oncology (SOHO 2022).

The research team conducted a cross-sectional structured survey of 28 oncologists in community-based settings from August 2021 to November 2021. The clinicians provided information about 55 advanced SM patients with a mean age of 56 years with the aim of determining referral patterns, symptomatology at the first visit, diagnostic approaches, and disease management strategies.

The survey results revealed a wide variety of referring specialties, including primary care, immunology, dermatology, oncology, and rheumatology, among others. Comorbid conditions included cardiovascular disease, diabetes, gastroesophageal reflux disease, chronic obstructive pulmonary disease, and asthma.

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The oncologists surveyed reported that the most critical diagnostic strategy for a definitive advanced SM diagnosis was bone marrow biopsy.

Symptoms reported by the patients included gastrointestinal, cutaneous, musculoskeletal, and neuropsychiatric symptoms, ranging from pruritus and cutaneous mastocytosis to nausea, bloating, and diarrhea. Allergic reactions, anxiety, dyspnea, and fatigue were also often reported at the first visit to an oncologist.

The authors recommend improving general clinician awareness of the potential clinical presentations of advanced SM along with effective diagnostic strategies to allow early recognition of the disease and lessen delay in treatment initiation.

SOHO 2022 is taking place from September 28, 2022, to October 1, 2022, in Houston, Texas.

Reference

Sullivan E, Daddona C, Green T, Lambert H, Acker E, Smith R. MPN-453 Clinical presentation and diagnosis of advanced systemic mastocytosis (AdvSM) in a community oncology setting. Clin Lymphoma Myeloma Leuk. Published online September 21, 2022. doi:10.1016/S2152-2650(22)01460-4