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Smouldering systemic mastocytosis - Clinical Profile, Treatments & Trials | Kisho | Kisho

Rare Disease

Smouldering systemic mastocytosis

MONDO:0015557

Overview

Smouldering systemic mastocytosis is a type of systemic mastocytosis (SM). This clonal hematologic disease, with a slow progression, results in an accumulation of neoplastic mast cells in the visceral organs over time and patients present with splenomegaly, hypercellular marrow and, in some cases, urticaria pigmentosa-like skin lesions.

Treatments1 FDA approved

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

1 FDA-approved treatment is available for Smouldering systemic mastocytosis, including CROMOLYN SODIUM (GASTROCROM, approved 1996).

Approved Therapies

Brand Name	Generic Name	Mechanism	Approved	Market Status

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for Smouldering systemic mastocytosis.

11 publications have been identified in PubMed for Smouldering systemic mastocytosis. Research spans Basic Science / Preclinical (27%), Epidemiology / Natural History (27%), and Review / Meta-Analysis (18%).

Research Type	Count	% of Total
Laboratory research	3	27%

Data assembled from 4 of 12 sources · Last updated Sep 21, 2026, 12:14 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:158775

GARD

Genetic and Rare Diseases Info Center

GARD:20036

Questions for Your Care Team

→What are the benefits and potential side effects of GASTROCROM for managing Smouldering systemic mastocytosis?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

Smouldering systemic mastocytosis

Overview

Treatment

Approved Therapies

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications

Smouldering systemic mastocytosis

Overview

Treatment

Approved Therapies

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications