indolent systemic mastocytosis
MONDO:0020331Mondo
Findings
No curated finding names indolent systemic mastocytosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Indolent systemic mastocytosis (ISM) is a benign form of systemic mastocytosis (SM) characterized by an abnormal proliferation of mast cells either only in bone marrow or in numerous tissues.
Definition from the Mondo Disease Ontology (MONDO:0020331), read 2026-09-29. CC BY 4.0.
Features
20 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal bone marrow cell morphologyHPOHP:0005561
- Very frequent (80% to 99% of cases)
- Abnormal mast cell morphologyHPOHP:0100494
- Very frequent (80% to 99% of cases)
- Abnormal skin morphologyHPOHP:0011121
- Very frequent (80% to 99% of cases)
- Increased CD25+ mast cell proportionHPOHP:0031408
- Very frequent (80% to 99% of cases)
- MastocytosisHPOHP:0100495
- Very frequent (80% to 99% of cases)
- Abdominal crampsHPOHP:0032155
- Frequent (30% to 79% of cases)
- AllergyHPOHP:0012393
- Frequent (30% to 79% of cases)
- Darier's signHPOHP:0025081
- Frequent (30% to 79% of cases)
- Elevated total serum tryptaseHPOHP:0031901
- Frequent (30% to 79% of cases)
- FlushingHPOHP:0031284
- Frequent (30% to 79% of cases)
- Generalized abnormality of skinHPOHP:0011354
- Frequent (30% to 79% of cases)
- Maculopapular exanthemaHPOHP:0040186
- Frequent (30% to 79% of cases)
Reported absent (1)
- Hematological neoplasmHPOHP:0004377
Show the remaining 8
- OsteoporosisHPOHP:0000939
- Frequent (30% to 79% of cases)
- PruritusHPOHP:0000989
- Frequent (30% to 79% of cases)
- Skin rashHPOHP:0000988
- Frequent (30% to 79% of cases)
- UrticariaHPOHP:0001025
- Frequent (30% to 79% of cases)
- Anaphylactic shockHPOHP:0100845
- Occasional (5% to 29% of cases)
- HepatomegalyHPOHP:0002240
- Very rare (1% to 4% of cases)
Where it sits
- A kind of
- Narrower terms (2)
Other names
2 names
Resolves to: indolent systemic mastocytosis
- Also called
- indolent systemic mastocytosis (morphologic abnormality)ISM