aggressive systemic mastocytosis

ORPHA: 98850

Overview

Human disease

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with aggressive systemic mastocytosis, sourced from HPO and Orphanet clinical annotations.

Abnormal mast cell morphologyOsteoporosisPruritusHepatosplenomegalyWeight lossIncreased total leukocyte countDiarrheaMalabsorptionAbdominal painAnorexiaHypotensionBone painLymphadenopathyArthralgiaElevated circulating alkaline phosphatase concentrationFatigueConstitutional symptomFlushingIncreased proportion of CD25+ mast cellsElevated total serum tryptaseAbdominal crampsAnaphylactic shockUrticariaPortal hypertensionDecreased liver functionAscitesThrombocytopeniaDecreased total neutrophil countPancytopeniaAnemiaLeukemiaHypersplenismGastrointestinal hemorrhagePathologic fractureOsteolysisHematological neoplasmAbnormal blistering of the skinAbnormal skin morphologyMaculopapular exanthema

Classification & Codes

Orphanet Code

ORPHA:98850
aggressive systemic mastocytosis
OrphanetORPHA:98850
Treatments0 drug(s)
Symptoms on record39 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO