Midostaurin (3) – Rydapt®

Systemic Mastocytosis

Characteristics

Start date 15.11.2023 – Marketing authorisation: 18.09.2017
Resolution 02.05.2024
INN Midostaurin
Brand name Rydapt®
Pharm. company Novartis Pharma AG
G-BA Procedure ID D-992
ATC code L01EX10 Other protein kinase inhibitors (L01EX)
ICD-10 codes (AIS) C94.30Mast cell leukemia with failed remission, C94.31Mast cell leukemia, in remission, C96.2Malignant mast cell neoplasm
Alpha-ID codes (AIS) I116195Aggressive systemic mastocytosis, I18159Mast cell leukemia, I31132Mast cell leukemia in complete remission
ORPHAcodes (AIS) 98850Aggressive systemic mastocytosis, 98851Mast cell leukemia, 98851Mast cell leukemia in complete remission
Therapeutic area Oncological diseases Mastocytosis / Systemic mastocytosis Orphan (turnover limit)
Reason for procedure Reassessment: Orphan turnover exceeded
Original resolution: Midostaurin (1) (05.04.2018)
Specialty Bundling ACT change

Therapeutic indication of the resolution

Rydapt is used as monotherapy for the treatment of adult patients with aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematologic neoplasia (SM-AHN) or mast cell leukemia (MCL)

Subpopulation Indication Comparator
Adults with aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematologic neoplasia (SM-AHN) or mast cell leukemia (MCL) Patient-specific therapy with selection of – Avapritinib (only for people after at least one prior systemic therapy and with platelet counts ≥ 50 x 109/L), – cladribine and – Imatinib (only for people without KIT D816V mutation or with unknown KIT mutation status and for people with existing eosinophilia with FIP1L1-PDGFRA fusion gene), taking into account the general condition, KIT mutation status and previous therapy

Studies and Results

No. of studies
(best subpopulation)
0 (Data not accepted)
Study design
(best subpopulation)
Data not accepted (Dossier: Single-arm + historical comparison)
Reason for dividing into subpopulations (G-BA) Time of treatment
ACT change 28.11.2023 – Neue Therapiestandards

  • Clinical trials
    • The CPKC412D2201 and CPKC412A2213 trials are single-arm Phase II trials.
    • The CPKC412D2201 trial enrolled 116 patients with aggressive systemic mastocytosis or mast cell leukaemia.
    • The CPKC412A2213 trial included 26 patients with histologically confirmed ASM or MCL.

Adults with aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated haematological neoplasia (SM-AHN) or mast cell leukaemia (MCL)

  • An additional benefit is not proven.
  • Consequently, there are no suitable data available overall for assessing the additional benefit of midostaurin.
  • Consequently, no additional benefit of midostaurin has been demonstrated for adults with aggressive systemic mastocytosis (ASM), There is no proof that systemic mastocytosis with associated haematological neoplasia (SM-AHN) or mast cell leukaemia (MCL) compared with the appropriate comparator therapy is better than the comparator therapy.
  • Overall assessment
    • The results of the submitted single-arm studies CPK412D2201 and CPK412A2213 are not suitable for assessing additional benefit, as they do not allow for a comparison with the appropriate comparator therapy.
    • The results of Lübke’s non-randomised comparison are also not taken into account for the benefit assessment due to the lack of detailed information on methodology and the failure to implement the appropriate comparator therapy.

Courtesy translation only, please refer to the German original.

Associated procedures

Midostaurin (3) Rydapt® Novartis Pharma AG Oncological diseases Systemic Mastocytosis 300–400 100% additional benefit not proven Orphan (turnover limit)
Midostaurin (2) Rydapt® Novartis Pharma AG Oncological diseases Acute myeloic Leukemia, FLT3-Mutation 380–1,040 100% additional benefit not proven Orphan (turnover limit)
Midostaurin (1) Rydapt® Novartis Pharma GmbH Oncological diseases Acute myeloid leukaemia (AML); Systemic mastocytosis (ASM) 0
400–710
80% considerable additional benefit Orphan repealed


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