A new study has found that organomegalies, including lymphadenopathy, are common in patients with systemic mastocytosis (SM) and that new occurrences of organomegaly were associated with disease progression and poorer outcomes.

The study was published in the Journal of Allergy and Clinical Immunology: In Practice.

“Although organomegaly (splenomegaly, hepatomegaly, lymphadenopathy) is a common feature in patients with smoldering SM and advanced SM [AdvSM], previous studies about the potential relevance of organomegaly were often limited to smaller cohorts and a more detailed investigation of prevalence and prognostic impact of organomegaly is still lacking,” the authors wrote. “We therefore sought to investigate the impact of organomegaly in a series of 3155 patients enrolled in the registry of the European Competence Network on Mastocytosis [ECNM].”

The research team analyzed the records of 3115 patients with cutaneous mastocytosis and SM from 2019 in the ECNM registry. All patients included had at least 1 entry for splenomegaly, hepatomegaly, and/or lymphadenopathy.  

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The analysis found organomegaly to be rare in indolent SM, cutaneous mastocytosis, and mastocytosis in the skin, but more common in cases of smoldering SM and AdvSM, within which the most common organomegaly was splenomegaly. The most aggressive disease phenotype and the poorest overall survival were found among patients with 3 organomegalies.

The analysis showed that patients with indolent SM and organomegaly had a similar progression and prognosis as those with smoldering SM.

The authors conclude that new occurrences of organomegaly can be associated with disease progression, and they emphasize the need for repeated physical and radiographic assessments during follow-up to be able to detect such cases of progression early. They recommend employing ultrasound in addition to physical examination to help with the diagnosis as well as with determining the underlying cause of the organomegaly, which will help guide treatment.

Reference

Lübke J, Schwaab J, Christen D et al. Prognostic impact of organomegaly in mastocytosis: an analysis of the European Competence Network on Mastocytosis. J Allergy Clin Immunol Pract. Published online November 16, 2022. doi:10.1016/j.jaip.2022.10.051