Mastocytosis

Leann Olansky, MD Reviewed 05/2023

BASICS

Mastocytosis is a rare, heterogeneous group of disorders caused by the abnormal clonal proliferation and accumulation of atypical mast cells in one or more organs of the body, most often affect...

DIAGNOSIS

Diagnosis is by identification of malignant mast cells by their morphology, immunophenotype, and/or molecular signature. WHO defined these criteria in 2001 and updated in 2016 most recently.
...

TREATMENT

Many of the first-line therapies are directed to symptom control. In recent years, new therapies have been designed for advanced SM and more are being actively investigated (2)[C].

Avoid en...

ONGOING CARE

Caution to avoid provocative stimuli.
If low bone density or osteoporosis identified on DXA scanning- use bisphosphonate therapy or denosumab.
Epinephrine auto-injector for anyone who has h...

REFERENCES

Hussein SE, Chifotides HT, Khoury JD, et al. Systemic mastocytosis and other entities involving mast cells: a practical review and update. Cancers (Basel). 2022;14(14):3474–3490. doi: 10.3...

ADDITIONAL READING

National Organization for Rare Disorders—Mastocytosis

CODES

ICD10

Q82.2 Mastocytosis
D47.0 Histiocytic and mast cell tumors of uncertain behavior
C96.2 Malignant mast cell tumor

SNOMED

397012002 cutaneous mastocytosis (disorder)
397016004 Systemic mast cell di...

CLINICAL PEARLS

Symptoms of mast cell activation can overshadow the cutaneous findings, which can be subtle and easily overlooked. The brownish papules and macules should be carefully sought.
When hypo...

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