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Dtsch Med Wochenschr 2006; 131(28/29): 1616-1621
DOI: 10.1055/s-2006-947809
Übersicht | Review article
Dermatologie, Allergologie
© Georg Thieme Verlag KG Stuttgart · New York

Mastozytose - klinisches Bild und Diagnostik

Mastocytosis - clinical findings and diagnosisF. Ruëff1 , S. Dugas-Breit1 , C. Bauer1 , M. Placzek1 , B. Przybilla1
  • 1Klinik und Poliklinik für Dermatologie und Allergologie der Universität München
Further Information

Publication History

eingereicht: 16.2.2006

akzeptiert: 23.6.2006

Publication Date:
06 July 2006 (online)

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Schlüsselwörter

Mastozytose - Osteoporose - Tryptase - Urticaria pigmentosa

Key words

mastocytosis - osteoporosis - tryptase - urticaria pigmentosa

Literatur

  • 1 Akin C, Fumo G, Yavuz A S, Lipsky P E, Neckers L, Metcalfe D D. A novel form of mastocytosis associated with a transmembrane c-kit mutation and response to imatinib.  Blood. 2004;  103 3222-3225
    Google Scholar
  • 2 Bauer C, Ruëff F, Przybilla B. Notfälle in der Allergologie.  Hautarzt. 2004;  55 525-532
    Google Scholar
  • 3 Blunk J A, Schmelz M, Zeck S, Skov P, Likar R, Koppert W. Opioid-induced mast cell activation and vascular responses is not mediated by mu-opioid receptors: an in vivo microdialysis study in human skin.  Anesth Analg. 2004;  98 364-370
    Google Scholar
  • 4 Bucher B, Simic P, Furrer J, Wüthrich B. Mastozytose: eine wichtige Differentialdiagnose bei anaphylaktischer Reaktion auf Hymenopterenstiche.  Schweiz Rundsch Med Prax. 2000;  89 411-418
    Google Scholar
  • 5 Brockow K, Scott L M, Worobec A S, Kirshenbaum A, Akin C, Huber M M, Metcalfe D D. Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease.  Arch Dermatol. 2002;  138 785-790
    Google Scholar
  • 6 Brockow K, Akin C, Huber M, Metcalfe D D. Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathology.  J Am Acad Dermatol. 2003;  48 508-516
    Google Scholar
  • 7 Czarnetzki B M, Kolde G, Schoemann A, Urbanitz S, Urbanitz D. Bone marrow findings in adult patients with urticaria pigmentosa.  J Am Acad Dermatol. 1988;  18 45-51
    Google Scholar
  • 8 Delling G, Ritzel H, Werner M. Histologische Charakteristika und Häufigkeit der sekundären Osteoporose bei systemischer Mastozytose. Eine retrospektive Analyse an 158 Fällen.  Pathologe. 2001;  22 132-140
    Google Scholar
  • 9 Dugas-Breit S, Schopf P, Dugas M, Schiffl H, Rueff F, Przybilla B. Baseline serum levels of mast cell tryptase are raised in hemodialysis patients and associated with severity of pruritus.  J Dtsch Dermatol Ges. 2005;  3 343-347
    Google Scholar
  • 10 Fearfield L A, Francis N, Henry K, Costello C, Bunker C B. Bone marrow involvement in cutaneous mastocytosis.  Br J Dermatol. 2001;  144 561-566
    Google Scholar
  • 11 Florian S, Krauth M T, Simonitsch-Klupp I, Sperr W R, Fritsche-Polanz R, Sonneck K, Fodinger M, Agis H, Bohm A, Wimazal F, Horny H P, Valent P. Indolent systemic mastocytosis with elevated serum tryptase, absence of skin lesions, and recurrent severe anaphylactoid episodes.  Int Arch Allergy Immunol. 2005;  136 273-280
    Google Scholar
  • 12 Fricker M, Helbling A, Schwartz L, Müller U. Hymenoptera sting anaphylaxis and urticaria pigmentosa: clinical findings and results of venom immunotherapy in ten patients.  J Allergy Clin Immunol. 1997;  100 11-15
    Google Scholar
  • 13 Golkar L, Bernhard J D. Mastocytosis.  Lancet. 1997;  349 1379-1385
    Google Scholar
  • 14 Hartmann K, Metcalfe D D. Pediatric mastocytosis.  Hematol Oncol Clin North Am. 2000;  14 625-640
    Google Scholar
  • 15 Hidvegi B, Nagy E, Szabo T, Temesvari E, Marschalko M, Karpati S, Horvath A, Gergely P. Correlation between T-cell and mast cell activity in patients with chronic urticaria.  Int Arch Allergy Immunol. 2003;  132 177-182
    Google Scholar
  • 16 Horan R F, Austen K F. Systemic mastocytosis: retrospective review of a decade’s clinical experience at the Brigham and Women’s Hospital.  J Invest Dermatol. 1991;  96 5S-14S
    Google Scholar
  • 17 Kettelhut B V, Metcalfe D D. Pediatric mastocytosis.  Ann Allergy. 1994;  73 197-202
    Google Scholar
  • 18 Kiszewski A E, Duran-Mckinster C, Orozco-Covarrubias L, Gutierrez-Castrellon P, Ruiz-Maldonado R. Cutaneous mastocytosis in children: a clinical analysis of 71 cases.  J Eur Acad Dermatol Venereol. 2004;  18 285-290
    Google Scholar
  • 19 Kors J W, Van Doormaal J J, Breukelman H, Van Voorst Vader P C, De Monchy J G. Long-term follow-up of indolent mastocytosis in adults.  J Intern Med. 1996;  239 157-164
    Google Scholar
  • 20 Kränke B, Sturm G, Aberer W. Negative venom skin test results and mastocytosis.  J Allergy Clin Immunol. 2004;  113 180-181
    Google Scholar
  • 21 Lawrence J B, Friedman B S, Travis W D, Chinchilli V M, Metcalfe D D, Gralnick H R. Hematologic manifestations of systemic mast cell disease: a prospective study of laboratory and morphologic features and their relation to prognosis.  Am J Med. 1991;  91 612-624
    Google Scholar
  • 22 Ludolph-Hauser D, Ruëff F, Fries C, Schöpf P, Przybilla B. Constitutionally raised serum concentrations of mast-cell tryptase and severe anaphylactic reactions to Hymenoptera stings.  Lancet. 2001;  357 361-362
    Google Scholar
  • 23 Ludolph-Hauser D, Schöpf P, Ruëff F, Przybilla B. Okkulte kutane Mastozytose.  Hautarzt. 2001;  52 390-393
    Google Scholar
  • 24 Marone G, Stellato C, Mastronardi P, Mazzarella B. Mechanisms of activation of human mast cells and basophils by general anesthetic drugs.  Ann Fr Anesth Reanim. 1993;  12 116-125
    Google Scholar
  • 25 Middelkamp Hup M A, Heide R, Tank B, Mulder P G, Oranje A P. Comparison of mastocytosis with onset in children and adults.  J Eur Acad Dermatol Venereol. 2002;  16 115-120
    Google Scholar
  • 26 Möller R, Paul E. Acetylsalicylsäure als Augmentationsfaktor einer Nahrungsmittel-Allergie.  Hautarzt. 1996;  47 281-283
    Google Scholar
  • 27 Müller U, Helbling A, Hunziker T, Wuthrich B, Pecoud A, Gilardi S, Beretta E, Fasel J, Messerli W, Maurer P. Mastocytosis and atopy: a study of 33 patients with urticaria pigmentosa.  Allergy. 1990;  45 597-603
    Google Scholar
  • 28 Oude Elberink J NG, de Monchy J GR, Kors J W, van Doormaal J J, Dubois A EJ. Fatal anaphylaxis after a yellow jacket sting, despite venom immunotherapy, in two patients with mastocytosis.  J Allergy Clin Immunol. 1997;  99 153-154
    Google Scholar
  • 29 Pardanani A, Kimlinger T K, Reeder T L, Li C Y, Tefferi A. Differential expression of CD2 on neoplastic mast cells in patients with systemic mast cell disease with and without an associated clonal haematological disorder.  Br J Haematol. 2003;  120 691-694
    Google Scholar
  • 30 Pardanani A, Ketterling R P, Brockman S R, Flynn H C, Paternoster S F, Shearer B M, Reeder T L, Li C Y, Cross N C, Cools J, Gilliland D G, Dewald G W, Tefferi A. CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy.  Blood. 2003;  102 3093-3096
    Google Scholar
  • 31 Pardanani A. Systemic mastocytosis: bone marrow pathology, classification, and current therapies.  Acta Haematol. 2005;  114 41-51
    Google Scholar
  • 32 Przybilla B, Müller U, Jarisch R, Ruëff F. Erhöhte basale Serumtryptasekonzentration oder Mastozytose als Risikofaktor der Hymenopterengiftallergie. Leitlinie der Deutschen Gesellschaft für Allergologie und klinische Immunologie.  Allergo J. 2004;  3 440-442
    Google Scholar
  • 33 Raisz L. Pathogenesis of osteoporosis: concepts, conflicts, and prospects.  J Clin Invest. 2005;  115 3318-2225
    Google Scholar
  • 34 Rodermund O E, Klingmuller G, Rohner H G. Interne Befunde bei Mastozytose.  Hautarzt. 1980;  31 175-178
    Google Scholar
  • 35 Rosbotham J L, Malik N M, Syrris P, Jeffery S, Bedlow A, Gharraie S, Murday V A, Holden C A, Carter N D. Lack of c-kit mutation in familial urticaria pigmentosa.  Br J Dermatol. 1999;  140 849-852
    Google Scholar
  • 36 Ruëff F, Wenderoth A, Przybilla B. Patients still reacting to a sting challenge while receiving Hymenoptera venom immunotherapy are protected by increased venom doses.  J Allergy Clin Immunol. 2001;  108 1027-1032
    Google Scholar
  • 37 Ruëff F, Dugas-Breit S, Bauer C, Schöpf P, Albert K, Marx R, Przybilla B. Diagnose und Therapie der Insektengiftallergie bei Mastozytose.  Allergo J. 2005;  14 514-515
    Google Scholar
  • 38 Ruëff F, Bauer C, Albert K, Przybilla B. Mastocytosis is a stronger risk factor for treatment failure of Hymenoptera venom immunotherapy than elevated baseline serum tryptase.  Allergy Clin Immunol Int - J WAO Org. 2005;  (Suppl 1) 17 69
    Google Scholar
  • 39 Sagher F, Even-Paz Z. Mastocytosis and the mast cell: Chicago: Year Book Medical Publishers, 1967 (zitiert nach Golkar L, Bernhard JD. Mastocytosis).  Lancet. 1997;  349 1379-1385
    Google Scholar
  • 40 Schwartz L B, Metcalfe D D, Miller J S, Earl H, Sullivan T. Tryptase levels as an indicator of mast-cell activation in systemic anaphylaxis and mastocytosis.  N Engl J Med. 1987;  316 1622-1626
    Google Scholar
  • 41 Schwartz L B, Bradford T B, Rouse C, Irani A -M, Rasp G, van der Zwan J K, van der Linden P -WG. Development of a new, more sensitive immunoassy for human tryptase: use in systemic anaphylaxis.  J Clin Immunol. 1994;  14 190-204
    Google Scholar
  • 42 Schwartz L B, Sakai K, Bradford T R, Ren S, Zweimann B, Worobec A S. The alpha form of human tryptase is the predominant type present in blood at baseline in normal subjects and is elevated in those with systemic mastocytosis.  J Clin Invest. 1995;  96 2702-2710
    Google Scholar
  • 43 Siegert S I, Diebold J, Ludolph-Hauser D, Lohrs U. Are gastrointestinal mucosal mast cells increased in patients with systemic mastocytosis?.  Am J Clin Pathol. 2004;  122 560-565
    Google Scholar
  • 44 Sotlar K, Horny H P, Simonitsch I, Krokowski M, Aichberger K J, Mayerhofer M, Printz D, Fritsch G, Valent P. CD25 indicates the neoplastic phenotype of mast cells: a novel immunohistochemical marker for the diagnosis of systemic mastocytosis (SM) in routinely processed bone marrow biopsy specimens.  Am J Surg Pathol. 2004;  28 1319-1325
    Google Scholar
  • 45 Sperr W R, Stehberger B, Wimazal F, Baghestanian M, Schwartz L B, Kundi M, Semper H, Jordan J H, Chott A, Drach J, Jager U, Geissler K, Greschniok A, Horny H P, Lechner K, Valent P. Serum tryptase measurements in patients with myelodysplastic syndromes.  Leuk Lymphoma. 2002;  43 1097-1105
    Google Scholar
  • 46 Stankovic K, Sarrot-Reynauld F, Puget M, Massot C, Ninet J, Lorcerie B, Dupond J L, Salles G, Durieu I, Vital Durand D, Rousset H. Systemic mastocytosis: predictable factors of poor prognosis present at the onset of the disease.  Eur J Intern Med. 2005;  16 387-390
    Google Scholar
  • 47 Valent P, Akin C, Sperr W R, Mayerhofer M, Fodinger M, Fritsche-Polanz R, Sotlar K, Escribano L, Arock M, Horny H P, Metcalfe D D. Mastocytosis: pathology, genetics, and current options for therapy.  Leuk Lymphoma. 2005;  46 35-48
    Google Scholar
  • 48 Williams A, Krishna T, Frew A. Systemic mastocytosis.  Lancet. 2002;  360 1611
    Google Scholar

Priv.-Doz. Dr. Franziska Ruëff

Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München - Campus Innenstadt

Frauenlobstraße 9-11

80337 München

Phone: 0049/89/51606170

Fax: 0049/89/51606162

Email: Franziska.Rueff@med.uni-muenchen.de