Evaluation of an infant or child with MPCM/UP and elevated tryptase

The algorithm depicts the authors' approach to monitoring children with MPCM/UP and elevated tryptase. This evaluation can be performed gradually over time during which the patient is being followed clinically. Testing for HaT may explain the elevated tryptase level, and KIT mutation analysis of peripheral blood may help avoid a bone marrow evaluation. If concerning physical exam features or lab abnormalities (other than the elevated tryptase) develop at any time, or if tryptase levels increase, the patient's skin lesions persist into adolescence, or recurrent episodes of anaphylaxis begin to occur, then a bone marrow evaluation is indicated.

MPCM/UP: maculopapular cutaneous mastocytosis/urticaria pigmentosa; SM: systemic mastocytosis; HaT: hereditary alpha tryptasemia.
* The level of tryptase that is considered elevated is evolving. The World Health Organization diagnostic criteria have defined levels >20 ng/mL as a minor criterion for systemic mastocytosis. The authors of this topic consider this evaluation appropriate with levels >10 ng/mL. The reasoning for this difference is discussed in the text of the UpToDate topic on the clinical manifestations and diagnosis of mastocytosis in children.
¶ Physical exam features suggestive of more advanced (aggressive) forms of systemic mastocytosis include hepato- or splenomegaly, and unexplained lymphadenopathy. Concerning laboratory features include elevated liver function tests or persistent cytopenias or immature/abnormal myeloid and lymphoid leukocytes.
Δ Children with cutaneous disease and MPCM/UP typically have increasing numbers of skin lesions between the ages of one and seven years, and then the lesions become lighter and lessen in number.

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Evaluation of an infant or child with MPCM/UP and elevated tryptase