Although targeted therapy involving tyrosine kinase inhibitors (TKIs) such as imatinib mesylate is highly effective for gastrointestinal stromal tumor carrying V560G c-kit mutation, it does not show much potential for targeting wild-type KIT (WT-KIT).
Systemic mastocytosis with <i>KIT</i> V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome.
To the best of our knowledge this first report of a patient with ISM, whose bone marrow MCs carry the <i>KIT</i> V560G activating mutation, manifesting as recurrent spontaneous episodes of flushing and vascular collapse in the absence of skin lesions at the time of diagnosis, in whom disodium cromoglycate had led to long term clinical remission.