Smouldering mastocytosis: a novel subtype of systemic mastocytosis with slow progression

International Archives of Allergy and Immunology
P ValentD D Metcalfe

Abstract

Systemic mastocytosis (SM) is a clonal disease that shows an either indolent or an aggressive clinical course. Utilizing established criteria, indolent SM can readily be discriminated from the rare aggressive subvariants of SM in most cases. In a small group of patients, however, clinical and laboratory parameters are indicative of slow progression without signs of aggressive disease or an associated hemopoietic neoplasm. These SM patients exhibit a high burden of mast cells, hypercellular marrow and organomegaly. Because of the 'intermediate' course and uncertain prognosis, these cases have been referred to as smouldering SM. In the present article, we discuss clinical and laboratory findings in smouldering SM and review the current literature. In addition, the pathophysiology of this novel subtype of SM is discussed.

Citations

Oct 28, 2003·Clinical Nuclear Medicine·Jabour KhouryAdnan Zaina
Jan 30, 2004·Annual Review of Medicine·Cem Akin, Dean D Metcalfe
Apr 22, 2014·Immunology and Allergy Clinics of North America·Cem Akin, Peter Valent
Apr 8, 2014·Clinical and Experimental Allergy : Journal of the British Society for Allergy and Clinical Immunology·P Valent
May 23, 2012·Medicina clínica·Alberto Alvarez-LarránMaría Rozman
Aug 22, 2003·British Journal of Haematology·Peter ValentDean D Metcalfe
Sep 22, 2010·Blood·Peter ValentCem Akin
Aug 30, 2014·Expert Review of Hematology·Peter ValentMichel Arock
Feb 5, 2011·International Archives of Allergy and Immunology·Peter Valent, Hans-Peter Horny
Oct 4, 2018·American Journal of Hematology·Ayalew TefferiAnimesh Pardanani
Dec 29, 2004·Leukemia & Lymphoma·Peter ValentDean D Metcalfe
Nov 25, 2004·Hematology·Stephen O'BrienPeter Valent
Jun 21, 2019·International Journal of Molecular Sciences·Peter ValentMassimo Triggiani
Jul 12, 2012·Expert Review of Hematology·Wolfgang R Sperr, Peter Valent
Jan 31, 2017·Annual Review of Pathology·Dean D Metcalfe, Yoseph A Mekori
Apr 30, 2020·Frontiers in Pharmacology·Miguel Piris-Villaespesa, Ivan Alvarez-Twose
Oct 20, 2017·British Journal of Haematology·Robyn M Scherber, Uma Borate
May 28, 2008·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·José A MoroAntonio Salvador

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