SM-AHN is a recognised variant of systemic mastocytosis in which the patient simultaneously carries a distinct, concurrently documented hematologic neoplasm. Because two separate diseases are present, the treatment approach must account for both.
The patient fulfills full diagnostic criteria for systemic mastocytosis and independently fulfills the diagnostic criteria for an associated hematologic neoplasm — each diagnosis established in its own right.
A critical first step is determining which component — the SM or the associated hematologic neoplasm — is the more clinically urgent driver, as that assessment directs where initial therapy is aimed. The range of options available depends substantially on that determination.
Adequate response means resolution of organ-damage findings, a measurable reduction in bone marrow mast cell burden, and a reduction in serum tryptase level — with these improvements maintained or confirmed over at least 12 weeks.
DOI: 10.6004/jnccn.2018.0088