Decision to perform bone marrow biopsy in an adult with suspected systemic mastocytosis (SM) but WITHOUT mastocytosis in the skin (MIS)

REMA: Spanish Network on Mastocytosis (Red Española de Mastocitosis); BM: bone marrow; MIS: mastocytosis in the skin; SM: systemic mastocytosis; CBC: complete blood count.
* In adults with symptoms of mast cell activation but without MIS, validated clinical and laboratory criteria have been defined that predict the presence of clonal mast cells in the bone marrow or systemic mastocytosis. These criteria are used to generate a "REMA score" (named for the Spanish Network on Mastocytosis). For a full explanation of the REMA score, refer to the UpToDate topic on clinical manifestations and diagnosis of mastocytosis in adults.
¶ Follow clinically and with yearly tryptase and CBC with differential. Severe symptoms that are consistent with SM (eg, hypotension, severe GI symptoms), increasing tryptase levels, or changes in CBC would prompt re-evaluation and possibly earlier or repeat bone marrow evaluation.