Figure 5. ISM screening algorithm. The... | American Society of Hematology

Figure 5.

$ISM screening algorithm. The algorithm was designed based on individual as well as combined test accuracy in predicting ISM (see supplemental Table 3). In the SM subgroup of patients we presented, 4 of 94 had neither monomorphic MPCM or high-risk anaphylaxis (REMA ≥2). Patients with a history of CM should screened by physical examination for the monomorphic MPCM subtype. All patients with the monomorphic MPCM subtype should be offered a BMB and high-sensitivity KIT p.D816V PCR testing. Patients with a history of anaphylaxis should be screened using the REMA score which also requires measurement of BST. Those with anaphylaxis with an associated REMA score ≥2 with BST values ≥11.5 ng/mL should also be screened with tryptase genotyping. Those with REMA score ≥2 and an elevated BST based upon genotype should be offered a BMB and high-sensitivity KIT p.D816V PCR testing. Patients with a REMA score ≥2 and a BST <11.5 ng/mL should be offered a BMB and high-sensitivity KIT p.D816V PCR testing on a case-by-case basis depending on factors such as patient age, trigger of anaphylaxis (eg, flying hymenoptera), and number of episodes of anaphylaxis with a REMA score ≥2. ∗It should also be noted that patients with ISM may present with other risk factors such as pathologic fractures, flushing, and mast cell hyperplasia in extracutaneous tissues rather than monomorphic MPCM or anaphylaxis. Patients with these risk factors without a clearly-defined cause, should also be screened with tryptase genotyping if the BST is ≥11.5 ng/mL. The accuracy of this screening approach in patients with SM subtypes other than ISM is not known.$

ISM screening algorithm. The algorithm was designed based on individual as well as combined test accuracy in predicting ISM (see supplemental Table 3). In the SM subgroup of patients we presented, 4 of 94 had neither monomorphic MPCM or high-risk anaphylaxis (REMA ≥2). Patients with a history of CM should screened by physical examination for the monomorphic MPCM subtype. All patients with the monomorphic MPCM subtype should be offered a BMB and high-sensitivity KIT p.D816V PCR testing. Patients with a history of anaphylaxis should be screened using the REMA score which also requires measurement of BST. Those with anaphylaxis with an associated REMA score ≥2 with BST values ≥11.5 ng/mL should also be screened with tryptase genotyping. Those with REMA score ≥2 and an elevated BST based upon genotype should be offered a BMB and high-sensitivity KIT p.D816V PCR testing. Patients with a REMA score ≥2 and a BST <11.5 ng/mL should be offered a BMB and high-sensitivity KIT p.D816V PCR testing on a case-by-case basis depending on factors such as patient age, trigger of anaphylaxis (eg, flying hymenoptera), and number of episodes of anaphylaxis with a REMA score ≥2. ∗It should also be noted that patients with ISM may present with other risk factors such as pathologic fractures, flushing, and mast cell hyperplasia in extracutaneous tissues rather than monomorphic MPCM or anaphylaxis. Patients with these risk factors without a clearly-defined cause, should also be screened with tryptase genotyping if the BST is ≥11.5 ng/mL. The accuracy of this screening approach in patients with SM subtypes other than ISM is not known.

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