Smoldering systemic mastocytosis Q82.2

In smoldering (systemic) mastocytosis, there is no skin involvement. The mast cell infiltrates are found exclusively in the bone marrow.

The original term "mastocytosis syndrome" was used for (systemic) mastocytosis in which no skin lesions were detectable. The term is no longer listed in more recent classifications.

In systemic mastocytoses (indolent systemic mastocytosis, aggressive systemic mastocytosis, mast cell leukemia), the skin is involved to varying degrees and frequency.

Irrespective of their tryptase level, patients complain, in addition to pruritus, of non-specific symptoms such as gastrointestinal complaints (diarrhoea, abdominal cramps, nausea, vomiting), tachycardia, hypotension, cephalgia, muscle pain, bone pain, flushing, fatigue and diffuse neuropsychiatric disorders.

Up to 30% of patients have bone involvement (osteopenia, osteoporosis, osteosclerosis, The osseous cytokines RANKL (receptor activator of nuclear factor kappa B-ligand), OPG (osteoprotegerin) and SOST (sclerostin) are elevated in patients with ISM(indolent systemic mastocytosis) and may interfere with bone remodeling.