Cutaneous manifestations in patients with mastocytosis: consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy …

K Hartmann, L Escribano, C Grattan, K Brockow… - Journal of Allergy and …, 2016 - Elsevier
Cutaneous lesions in patients with mastocytosis are highly heterogeneous and encompass
localized and disseminated forms. Although a classification and criteria for cutaneous …

Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria

P Valent, C Akin, L Escribano… - European journal of …, 2007 - Wiley Online Library
Although a classification for mastocytosis and diagnostic criteria are available, there remains
a need to define standards for the application of diagnostic tests, clinical evaluations, and …

Safety and efficacy of avapritinib in advanced systemic mastocytosis: the phase 1 EXPLORER trial

DJ DeAngelo, DH Radia, TI George, WA Robinson… - Nature medicine, 2021 - nature.com
Advanced systemic mastocytosis (AdvSM) is a rare hematologic neoplasm driven by the KIT
D816V mutation and associated with poor survival. This phase 1 study (NCT02561988) …

Paediatric mastocytosis: a systematic review of 1747 cases

C Méni, J Bruneau, S Georgin‐Lavialle… - British Journal of …, 2015 - academic.oup.com
Paediatric mastocytosis was previously considered to be a benign and spontaneously
regressing disease. However, this evolution is impossible to predict. To clarify the …

[HTML][HTML] Pediatric mastocytosis is a clonal disease associated with D816V and other activating c-KIT mutations

C Bodemer, O Hermine, F Palmérini, Y Yang… - Journal of Investigative …, 2010 - Elsevier
Adult mastocytosis is an incurable clonal disease associated with c-KIT mutations, mostly in
exon 17 (D 816 V). In contrast, pediatric mastocytosis often spontaneously regresses and is …

Structure and regulation of Kit protein-tyrosine kinase—the stem cell factor receptor

R Roskoski Jr - Biochemical and biophysical research communications, 2005 - Elsevier
Signaling by stem cell factor and Kit, its receptor, play important roles in gametogenesis,
hematopoiesis, mast cell development and function, and melanogenesis. Moreover, human …

International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study

WR Sperr, M Kundi, I Alvarez-Twose… - The Lancet …, 2019 - thelancet.com
Background The WHO classification separates mastocytosis into distinct variants, but
prognostication remains a clinical challenge. The aim of this study was to improve …

Mast cell leukemia

S Georgin-Lavialle, L Lhermitte… - Blood, The Journal …, 2013 - ashpublications.org
Mast cell leukemia (MCL) is a very rare form of aggressive systemic mastocytosis accounting
for< 1% of all mastocytosis. It may appear de novo or secondary to previous mastocytosis …

Proposed diagnostic algorithm for patients with suspected mastocytosis: a proposal of the European Competence Network on Mastocytosis

P Valent, L Escribano, S Broesby‐Olsen, K Hartmann… - Allergy, 2014 - Wiley Online Library
Mastocytosis is an emerging differential diagnosis in patients with more or less specific
mediator‐related symptoms. In some of these patients, typical skin lesions are found and the …

5. IgE, mast cells, basophils, and eosinophils

C Prussin, DD Metcalfe - Journal of allergy and clinical immunology, 2006 - Elsevier
IgE, mast cells, basophils, and eosinophils constitute essential elements in allergic
inflammation. Allergen-specific IgE, synthesized in response to allergens in the environment …