 |
 |
 |
|
 |
 |
 |
|
|
|
|
|
|
Mast Cell Disease
Mast cell disease, or mastocytosis, comprises a heterogeneous group of diseases characterized by an overabundance of active mast cells that accumulate in one or more organ systems.1
The World Health Organization (WHO) has classified mast cell disease into 7 distinct types.1 Broadly speaking, however, there are 2 major categories of disease: cutaneous mastocytosis and systemic mastocytosis. The majority (55%) of cases of mast cell disease occur in children under 2 years of age, and are the cutaneous form with little or no internal organ involvement.2,3 Cases beginning during adulthood tend to involve the inner organs—including the liver, spleen, and lymph nodes in addition to the skin.2,3
WHO Classification of Mast Cell Disease (Mastocytosis)1
- Cutaneous mastocytosis
- Indolent systemic mastocytosis
- Systemic mastocytosis with associated clonal, hematologic non-mast cell lineage disease
- Aggressive systemic mastocytosis
- Mast cell leukemia
- Mast cell sarcoma
- Extracutaneous mastocytoma
Epidemiology
The annual incidence of mast cell disease has been estimated at 2 persons per year in a population of 300,000, with both sexes equally affected.4
Most cases (55%) of mast cell disease occur in children under 2 years of age and are the cutaneous form. Adults account for 35% of cases of systemic mastocytosis, and these patients usually present between the ages of 20 and 40 years.4 Adult-onset mast cell disease is most often systemic.3
References:
- Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood. 2002;100:2292-2302.
- National Organization for Rare Diseases. Mastocytosis. Available at: http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Mastocytosis. Accessed April 26, 2006.
- Cortes J, Kantarjian H. Beyond chronic myelogenous leukemia: potential role for imatinib in Philadelphia-negative myeloproliferative disorders. Cancer. 2004;100:2064-2078.
- Hartmann K, Henz BM. Mastocytosis: recent advances in defining the disease. Br J Dermatol. 2001;144:682-695.
|
|
|
|
|
|
