Indolent systemic mastocytosis has a low disease progression rate, normal life expectancy in most adults

A prevalence of mastocytosis of 1 in 10,000 inhabitants has been reported, but underdiagnosis is assumed

Indolent systemic mastocytosis is a group of rare diseases for which reliable predictors of progression and outcome are still lacking.

Analysis showed that serum beta2-microglobulin and mast/stem cell growth factor receptor gene (KIT) mutation in mast cells plus myeloid and lymphoid hematopoietic lineages was the best combination for predicting disease progression.

Mast cells. Image source: Wikipedia.

The authors concluded that indolent systemic mastocytosis in adults has a low disease progression rate, and the great majority of patients have a normal life expectancy.

The presence of KIT mutation in all hematopoietic lineages and increased serum beta2-microglobulin are the most powerful independent parameters for predicting transformation into a more aggressive form of the disease.


Prognosis in adult indolent systemic mastocytosis: A long-term study of the Spanish Network on Mastocytosis in a series of 145 patients. Escribano L, Alvarez-Twose I, Sánchez-Muñoz L, Garcia-Montero A, Núñez R, Almeida J, Jara-Acevedo M, Teodósio C, García-Cosío M, Bellas C, Orfao A. J Allergy Clin Immunol. 2009 Jun 19.
Cutaneous mastocytosis in a 3-month-old girl
Neuropeptide blood levels correlate with mast cell load in patients with mastocytosis
Mastocytosis - Where are we now? World Allergy Organization summary, 2012.
Proposed diagnostic algorithm for patients with suspected mastocytosis. Allergy, 2014

Mast Cell Disorders (presentation on Google drive):

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