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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

1
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]
2
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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