A single-center retrospective study found differences in survival among patients with different types of mastocytosis and identified factors contributing to survival and prognosis. The study was recently published in BMC Cancer.
The study’s authors observed a negative correlation between survival and mast cell infiltration rate in the bone marrow, bone marrow dysplasia, and the occurrence of complications during the follow-up. Moreover, they identified the World Health Organization classification and the International Prognostic Scoring System in Mastocytosis (IPSM), which were used to evaluate survival, as indicators of prognosis.
The analysis showed that patients with advanced systemic mastocytosis (SM) had the worst survival rate, while patients with cutaneous mastocytosis had the better survival rate. Also, patients with a low IPSM score had longer survival than those with advanced SM with 2 or more risk factors.
At disease onset, patients mostly presented with skin lesions (63.5%), followed by anaphylaxis (19.2%). The most common symptoms included skin alterations (76%), hepatomegaly (32.7%), anaphylaxis (28.9%), and bone symptoms (28.9%).
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“The wide range of complaints may cause patients to consult various clinics, with resulting mis- or underdiagnosis. Therefore, cooperation with different branches in an excellence center plays an important role in the diagnosis and treatment of the disease,” the study’s authors said.
The study enrolled 104 patients (median age at onset, 35 years; 47.12% female), mostly diagnosed with SM (82.7%), including indolent SM (52.9%), SM with an associated hematologic (non-mast cell lineage) neoplasm (13.5%), smoldering SM (5.8%), aggressive SM (5.8%), and mast cell leukemia (4.8%). Most patients were positive for tryptase (81.7%), CD117 (82.5%), CD30 (55.8%), and C-KIT D816V (85.7%). Most patients were treated with H1 antagonists (94.23%) and H2 blockers (92.30%).
Eighteen (17.5%) patients experienced complications. Fourteen (16.3%) patients had a bone marrow mast cell percent above 25%, 86 (82.7%) patients had mast cell aggregates, and 15 (14.4%) patients had dysplasia.
Tiryaki TO, Özkan SG, Erdem S, et al. Comprehensive mastocytosis data analysis from a single center. BMC Cancer. 2023;23(1):82. doi:10.1186/s12885-022-10498-3