Correspondence
Mastocytosis – A Disease of the Hematopoietic Stem Cell: Problematic Criteria
Dtsch Arztebl Int 2009; 106(10): 173. DOI: 10.3238/arztebl.2009.0173
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From an internist's perspective, a diagnostic approach seems more appropriate in which the pathologically altered mast cell with its pathologically increased mediator release is the main criterion for a diagnosis of systemic mastocytosis. The symptoms of the mast cell mediator syndrome can be reliably detected in a standardized manner with an internationally validated questionnaire (for example, those from the Association Française pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses) (3).
When the presence of disorders is excluded in which normal mast cells may be activated, the occurrence of pathological excess production and release of mast cell mediators can only be a manifestation of pathological, uncontrolled, hyperactive mast cells. For this reason, the disorder is appropriately termed as the systemic mast cell hyperactivity syndrome and, in terms of terminology, is a systemic mastocytosis. If the pathologically increased mast cell activity is regarded as main diagnostic criterion, systematic mastocytoses are by no means rare disorders but very likely to be underdiagnosed. DOI: 10.3238/arztebl.2009.0173
On behalf of the the research group for systemic mast cell disorders, Bonn
Prof. Dr. med. Jürgen Homann
Evangelische Kliniken Bonn
Betriebsstätte Waldkrankenhaus
CA der Inneren Medizin
Waldstr. 73
53177 Bonn, Germany
Juergen.Homann@ek-bonn.de
Ulrich Kolck
Evangelische Kliniken Bonn
Betriebsstätte Waldkrankenhaus
OA der Kardiologie
Waldstr. 73
53177 Bonn, Germany
Ulrich.Kolck@ek-bonn.de
Prof. Dr. med. Gerhard J. Molderings
Institut für Humangenetik
Universitätsklinikum Bonn
Wilhelmstr. 31
53111 Bonn, Germany
molderings@uni-bonn.de