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Fortgeschrittene systemische Mastozytose (AdvSM)

Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.

IDDIS-MASTOCYTOSIS
ArtErkrankung
Aliase
ASM (aggressive systemische Mastozytose)AdvSMFortgeschrittene systemische MastozytoseMCL (Mastzellleukämie)SM-AHNПоширений системний мастоцитоз
Statusreviewed 2026-04-27 | pending_clinical_signoff
ErkrankungenNone declared
QuellenSRC-ESMO-AML-2020 SRC-NCCN-AML-2025 SRC-NCCN-SM-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Quelle: YAML

icd_o_3_morphology9740/3
icd_o_3_topography["C42.1"]
icd_10C96.2, D47.09
who_classificationWHO Hämatomymphoid 5 ed (2022): Systemische Mastozytose mit Subtypen (ISM / SSM / SM-AHN / ASM / MCL)

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Only ADVANCED forms get targeted therapy. WHO 2022 subtypes: - Indolent SM (ISM): KIT D816V+; symptomatic management (anti-mediator: H1/H2 blockers, montelukast, cromolyn, omalizumab); avapritinib (PIONEER) in moderate-severe symptomatic ISM despite optimal anti-mediator therapy. - Smoldering SM (SSM): borderline; observe with hybrid approach. - Aggressive SM (ASM): C-findings (organ damage from mast-cell infiltration) — targeted therapy indicated. - SM with associated hematologic neoplasm (SM-AHN): ~70% of advanced SM; AHN component (most often MDS / MPN / CMML; less often AML) drives prognosis; treat both compartments. - Mast cell leukemia (MCL): ≥20% mast cells in BM aspirate; very aggressive. Therapy for ASM / SM-AHN / MCL: - Avapritinib (EXPLORER + PATHFINDER): selective KIT D816V inhibitor; FDA-approved 2021 for advanced SM. Not effective for D816V-negative. Cognitive / cerebral microbleed signal at high dose — 200 mg/day. - Midostaurin (D-RIVE): multikinase including KIT — historically standard pre-avapritinib; ORR ~28%. - SM-AHN: treat AHN per its own histology — e.g. SM-AML with 7+3 + midostaurin if FLT3-ITD also present. - Allo-HSCT: consider in fit MCL or aggressive SM-...

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