Indolent Systemic Mastocytosis (ISM) with 0 or 1 B-Findings: Clinical Management

This protocol addresses systemic mastocytosis in patients classified as indolent systemic mastocytosis (ISM) with zero or one B-findings, or smoldering systemic mastocytosis (SSM) with two or more B-findings — in each case without C-findings and without an associated hematologic neoplasm.

Clinical Scenario

ISM is characterized by low mast cell burden, no evidence of C-findings, and no associated hematologic neoplasm (AHN). SSM is defined by two or more B-findings and likewise no evidence of C-findings or an AHN. The absence of organ damage and the lower disease burden in this group define the management approach.

Treatment Overview

For asymptomatic patients in this setting, the approach centers on active surveillance combined with patient counseling — addressing recognition of disease signs and symptoms and avoidance of known triggers of mast cell activation. Preparedness for anaphylaxis is a core component of management for all patients. Clinical trial enrollment may also be considered.

The complete structured protocol — including full management options for symptomatic presentations — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

ISM is characterized by low mast cell burden, no evidence of C-findings, or an AHN.
SSM is defined by ≥2 B-findings and no evidence of C-findings or an AHN.
All patients should carry 2 auto injectors of epinephrine to manage anaphylaxis.

DOI: 10.6004/jnccn.2018.0088

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