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Mastocytosis

Reviewed 06/2018
 


BASICS

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

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 2008.

  • To diagnose SM...

TREATMENT

Currently no cure and favorable prognosis, so management goals are for symptom relief (2
  • Avoid environmental triggers of mast cell degranulation (hymenoptera venom, extreme heat or temperat...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Depends on type of SM, coexisting disorders, severity of mediator-related symptoms, and therapy

  • Most children with ISM will continue to have ISM throughout their l...

REFERENCES

1
Azaña JM, Torrelo A, Matito A. Update on mastocytosis (part 1): pathophysiology, clinical features, and diagnosis. Actas Dermosifiliogr.  2016;107(1):5–14. [View Abstract on OvidMedli...

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 in cases ...

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