SM-AHN: Treatment When Initial Therapy Has Not Achieved Adequate Response
Clinical Scenario
This protocol addresses systemic mastocytosis with an associated hematologic neoplasm (SM-AHN) — a presentation that simultaneously fulfills the diagnostic criteria for systemic mastocytosis and for a separate, co-occurring associated hematologic neoplasm.
Prior Line — Inadequate Response
The preceding treatment approach for SM-AHN — which may have included AHN-directed therapy with concurrent SM management, clinical trial participation, midostaurin, cladribine, interferons ± prednisone, or allogeneic hematopoietic cell transplant — did not meet the required response threshold:
Adequate response with resolution of organ damage findings, reduction in bone marrow mast cell burden, and reduction in serum tryptase level — sustained and confirmed for at least 12 weeks.
This protocol defines the next step after that failure.
Next-Line Approach (partial overview)
The approach depends on whether the AHN component has shown progression. Disease-directed therapy and evaluation for transplant-based consolidation are among the considerations in this next line. Specific options, eligibility criteria, and treatment sequence are detailed in the full protocol.
Complete regimen — specific agents, selection criteria, and clinical algorithm — is available in the structured protocol below.
References
DOI: 10.6004/jnccn.2018.0088
- SM-AHN fulfills the diagnostic criteria for SM as well as the diagnostic criteria for the AHN.
- Patients with advanced SM with inadequate response or loss of response should be treated with alternate cytoreductive therapy not previously received.
- Evaluation of allogeneic HCT should be considered for patients with advanced SM (ASM, SM-AHN, or MCL) with adequate response to cytoreductive therapy and with suitable donor(s) identified.
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