This protocol covers patients diagnosed with mast cell leukemia (MCL) — characterised by ≥20% neoplastic mast cells on a bone marrow aspirate, with or without an associated hematologic neoplasm — who have already undergone first-line cytoreductive therapy and did not meet the defined response criteria.
First-line cytoreductive therapy — including clinical trial, midostaurin, or cladribine — aimed to achieve adequate response: resolution of organ damage findings, substantial reduction in bone marrow mast cell burden, and a sustained reduction in serum tryptase level maintained for at least 12 weeks. When these goals are not reached, escalation to the next protocol is indicated.
The next step involves re-staging and evaluation for allogeneic hematopoietic cell transplant (HCT). In the setting of associated hematologic neoplasm progression or transformation, a distinct category of systemic therapy is considered alongside concurrent MCL management. The full decision algorithm, eligibility conditions, and sequencing are contained in the complete protocol.
MCL is defined histopathologically by the presence of ≥20% neoplastic mast cells on a bone marrow aspirate.
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.
DOI: 10.6004/jnccn.2018.0088
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