Mastocytosis

Keren Zhou, MD and Leann Olansky, MD Reviewed 06/2019

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 (4)[C].

Avoid env...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Depends on type of SM, coexisting disorders, severity of mediator-related symptoms, and therapy
Adult patients with more severe mediator-related symptoms or high d...

REFERENCES

Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017;129(11):1420–1427. [View Abstract on OvidMedline]

Theo...

CODES

ICD10

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

ICD9

757.33 Congenital pigmentary anomalies of skin
202.60 Malignant mast cell tum...

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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