Systemic mastocytosis
ICD-10 D47.0 · ICD-11 2A21.0

Treatment of Indolent or Smoldering Systemic Mastocytosis with Recurrent Hypotension

Clinical Scenario

This protocol applies to patients with indolent (ISM) or smoldering (SSM) systemic mastocytosis who experience recurrent hypotensive episodes — a presentation indicating that standard symptom-directed measures are insufficient and further escalation is warranted.

Condition Context

In indolent and smoldering systemic mastocytosis, management is primarily directed toward anaphylaxis prevention, symptom control, and osteoporosis treatment. Recurrent hypotension signals a failure of these goals and drives the need for a more targeted intervention strategy.

Treatment Approach

When recurrent hypotension persists despite earlier management, the protocol specifies a cytoreductive therapy as the designated next step. The specific agent or agents, selection criteria, and clinical sequencing are defined in the full structured protocol.

The complete regimen — agent selection, dosing parameters, and decision criteria — is available in the structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1002/ajh.26962

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