Smouldering Mastocytosis: A Novel Subtype of Systemic Mastocytosis with Slow ProgressionValent P. · Akin C. · Sperr W.R. · Horny H.-P. · Metcalfe D.D.
aDepartment of Internal Medicine I, Division of Hematology, University of Vienna, Austria; bLaboratory of Allergic Diseases, NIH, Bethesda, Md., USA; cInstitute of Pathology, Medical University of Lübeck, Germany
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.
Correspondence to: Dr. Peter Valent
Department of Internal Medicine I, Division of Hematology and Hemostaseology
University of Vienna, Währinger Gürtel 18–20
A–1090 Vienna (Austria)
Tel. +43 1 40400 6085, Fax +43 1 402 6930, E-Mail email@example.com
Number of Print Pages : 3
Number of Figures : 0, Number of Tables : 2, Number of References : 19
International Archives of Allergy and Immunology
Vol. 127, No. 2, Year 2002 (Cover Date: February 2002)
Journal Editor: D. Kraft, Vienna
ISSN: 1018–2438 (print), 1423–0097 (Online)
For additional information:http://www.karger.com/journals/iaa