Universität zu Köln

Valent, P., Escribano, L., Broesby-Olsen, S., Hartmann, K., Grattan, C., Brockow, K., Niedoszytko, M., Nedoszytko, B., Elberink, J. N. G. Oude, Kristensen, T., Butterfield, J. H., Triggiani, M., Alvarez-Twose, I., Reiter, A., Sperr, W. R., Sotlar, K., Yavuz, S., Kluin-Nelemans, H. C., Hermine, O., Radia, D., van Doormaal, J. J., Gotlib, J., Orfao, A., Siebenhaar, F., Schwartz, L. B., Castells, M., Maurer, M., Horny, H. -P., Akin, C., Metcalfe, D. D. and Arock, M. (2014). Proposed diagnostic algorithm for patients with suspected mastocytosis: a proposal of the European Competence Network on Mastocytosis. Allergy, 69 (10). S. 1267 - 1275. HOBOKEN: WILEY. ISSN 1398-9995

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Abstract

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 diagnosis of mastocytosis can be established. In other cases, however, skin lesions are absent, which represents a diagnostic challenge. In the light of this unmet need, we developed a diagnostic algorithm for patients with suspected mastocytosis. In adult patients with typical lesions of mastocytosis in the skin, a bone marrow (BM) biopsy should be considered, regardless of the basal serum tryptase concentration. In adults without skin lesions who suffer from mediator-related or other typical symptoms, the basal tryptase level is an important parameter. In those with a slightly increased tryptase level, additional investigations, including a sensitive KIT mutation analysis of blood leucocytes or measurement of urinary histamine metabolites, may be helpful. In adult patients in whom (i) KIT D816V is detected and/or (ii) the basal serum tryptase level is clearly increased (>25-30ng/ml) and/or (iii) other clinical or laboratory features suggest the presence of occult' mastocytosis or another haematologic neoplasm, a BM investigation is recommended. In the absence of KIT D816V and other signs or symptoms of mastocytosis or another haematopoietic disease, no BM investigation is required, but the clinical course and tryptase levels are monitored in the follow-up. In paediatric patients, a BM investigation is usually not required, even if the tryptase level is increased. Although validation is required, it can be expected that the algorithm proposed herein will facilitate the management of patients with suspected mastocytosis and help avoid unnecessary referrals and investigations.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Valent, P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Escribano, L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Broesby-Olsen, S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hartmann, K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Grattan, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Brockow, K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Niedoszytko, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Nedoszytko, B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Elberink, J. N. G. Oude UNSPECIFIED UNSPECIFIED UNSPECIFIED Kristensen, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Butterfield, J. H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Triggiani, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Alvarez-Twose, I. UNSPECIFIED UNSPECIFIED UNSPECIFIED Reiter, A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sperr, W. R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sotlar, K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Yavuz, S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kluin-Nelemans, H. C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hermine, O. UNSPECIFIED UNSPECIFIED UNSPECIFIED Radia, D. UNSPECIFIED UNSPECIFIED UNSPECIFIED van Doormaal, J. J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Gotlib, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Orfao, A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Siebenhaar, F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schwartz, L. B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Castells, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Maurer, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Horny, H. -P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Akin, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Metcalfe, D. D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Arock, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-427798
DOI:	10.1111/all.12436
Journal or Publication Title:	Allergy
Volume:	69
Number:	10
Page Range:	S. 1267 - 1275
Date:	2014
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1398-9995
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MAST-CELL ACTIVATION; KIT D816V MUTATION; SYSTEMIC MASTOCYTOSIS; SERUM TRYPTASE; PROLIFERATIVE DISORDERS; CLASSIFICATION; ANAPHYLAXIS; DELINEATION; CRITERIA Multiple languages Allergy; Immunology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/42779