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Original Paper

Indolent Systemic Mastocytosis with Elevated Serum Tryptase, Absence of Skin Lesions, and Recurrent Severe Anaphylactoid Episodes

Florian S.a · Krauth M.-T.a · Simonitsch-Klupp I.b · Sperr W.R.a · Fritsche-Polanz R.c · Sonneck K.a · Födinger M.c · Agis H.a · Böhm A.a · Wimazal F.a · Horny H.-P.d · Valent P.a

Author affiliations

aDepartment of Internal Medicine I, Division of Hematology and Hemostaseology; bDepartment of Clinical Pathology, and cClinical Institute of Medical and Chemical Laboratory Diagnostics, Medical University of Vienna, Vienna, Austria, and dInstitute of Pathology, University of Lübeck, Lübeck, Germany

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Int Arch Allergy Immunol 2005;136:273–280

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: July 26, 2004
Accepted: October 11, 2004
Published online: March 16, 2005
Issue release date: March 2005

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 4

ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)

For additional information: https://www.karger.com/IAA

Abstract

Background: In contrast to aggressive mastocytosis, patients with indolent systemic mastocytosis (ISM) usually present with urticaria pigmentosa-like skin lesions. In those who lack skin lesions, mastocytosis is often overlooked or confused with endocrinologic, allergic, or other internal disorders. Case Report and Results: We report on a 33-year-old male patient in whom severe hypotensive episodes occurred after contact with ants or yellow jackets. Since no specific IgE was detected, the serum tryptase concentration was measured and found to be clearly elevated (70 ng/ml). Consecutive staging and examination of the bone marrow revealed ISM. The patient was advised to circumvent insect contact, to take antihistamines on demand, and to carry an epinephrine self-injector for emergency events. In a retrospective analysis of 40 patients seen between 1988 and 2003, only 2 had a life-threatening mediator-related episode before ISM was diagnosed. Conclusions: Our report confirms the diagnostic value of tryptase in patients with suspected mastocytosis. In addition, the report suggests that the lack of typical skin lesions does not exclude an indolent form of mastocytosis even if the serum tryptase is clearly elevated. Finally, our case further shows that mastocytosis can be an important differential diagnosis to be considered in patients with unexplained anaphylactoid or other mediator-related symptoms.

© 2005 S. Karger AG, Basel


References

  1. Lennert K, Parwaresch MR: Mast cells and mast cell neoplasia: A review. Histopathology 1979;3:349–365.
  2. Parwaresch MR, Horny HP, Lennert K: Tissue mast cells in health and disease. Pathol Res Pract 1985;179:439–361.
  3. Metcalfe DD: Classification and diagnosis of mastocytosis: Current status. J Invest Dermatol 1991;96:2S–4S.
    External Resources
  4. Valent P: Biology, classification and treatment of human mastocytosis. Wien Klin Wochenschr 1996;108:385–397.
  5. Caplan RM: The natural course of urticaria pigmentosa. Arch Dermatol 1963;87:146–149.
  6. Travis WD, Li CY, Bergstralh EJ, Yam LT, Swee RG: Systemic mast cell disease: Analysis of 58 cases and literature review. Medicine 1988;67:345–368.
  7. Akin C, Metcalfe DD: Systemic mastocytosis. Annu Rev Med 2004;55:419–432.
  8. Nagata H, Worobec AS, Oh CK, Chowdhury BA, Tannenbaum S, Suzuki Y, Metcalfe DD: Identification of a point mutation in the catalytic domain of the protooncogene c-kit in peripheral blood mononuclear cells of patients who have mastocytosis with an associated hematologic disorder. Proc Natl Acad Sci USA 1995;92:10560–10564.
  9. Longley BJ, Tyrrell L, Lu SZ, Ma YS, Langley K, Ding TG, Duffy T, Jacobs P, Tang LH, Modlin I: Somatic c-KIT activating mutation in urticaria pigmentosa and aggressive mastocytosis: Establishment of clonality in a human mast cell neoplasm. Nat Genet 1996;12:312–314.
  10. Büttner C, Henz BM, Welker P, Sepp NT, Grabbe J: Identification of activating c-kit mutations in adult-, but not in childhood-onset indolent mastocytosis: A possible explanation for divergent clinical behavior. J Invest Dermatol 1998;111:1227–1231.
  11. Fritsche-Polanz R, Jordan JH, Feix A, Sperr WR, Sunder-Plassmann G, Valent P, Födinger M: Mutation analysis of C-KIT in patients with myelodysplastic syndromes without mastocytosis and cases of systemic mastocytosis. Br J Haematol 2001;113:357–364.
  12. Horny HP, Parwaresch MR, Lennert K: Bone marrow findings in systemic mastocytosis. Hum Pathol 1985;16:808–814.
  13. Horny HP, Sillaber C, Menke D, Kaiserling E, Wehrmann M, Stehberger B, Chott A, Lechner K, Lennert K, Valent P: Diagnostic value of immunostaining for tryptase in patients with mastocytosis. Am J Surg Pathol 1998;22:1132–1140.
  14. Horny HP, Valent P: Diagnosis of mastocytosis: General histopathological aspects, morphological criteria, and immunohistochemical findings. Leuk Res 2001;25:543–551.
  15. Sperr WR, Escribano L, Jordan JH, Schernthaner GH, Kundi M, Horny HP, Valent P: Morphologic properties of neoplastic mast cells: Delineation of stages of maturation and implication for cytological grading of mastocytosis. Leuk Res 2001;25:529–536.
  16. Schwartz LB, Sakai K, Bradford TR, Ren S, Zweiman B, Worobec AS, Metcalfe DD: 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.
  17. Schwartz LB, Metcalfe DD, Miller JS, 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.
  18. Escribano L, Orfao A, Diaz-Agustin B, Villarrubia J, Cervero C, Lopez A, Marcos, MA, Bellas C, Fernandez-Canadas S, Cuevas M, Sanchez A, Velasco JL, Navarro JL, Miguel JF: Indolent systemic mast cell disease in adults: Immunophenotypic characterization of bone marrow mast cells and its diagnostic implications. Blood 1998;91:2731–2736.
  19. Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nunez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM: Diagnostic criteria and classification of mastocytosis: A consensus proposal. Leuk Res 2001;25:603–625.
  20. Valent P, Horny HP, Li CY, Longley JB, Metcalfe DD, Parwaresch RM, Bennett JM: Mastocytosis; in Jaffe ES, Harris NL, Stein H, Vardiman JW (eds): World Health Organization (WHO) Classification of Tumours. Pathology and Genetics. Tumours of Haematopoietic and Lymphoid Tissues. Oxford, Oxford University Press, 2001, vol 1, pp 291–302.
  21. Sperr WR, Jordan JH, Fiegl M, Escribano L, Bellas C, Dirnhofer S, Simonitsch-Klupp I, Horny HP, Valent P: Serum tryptase levels in patients with mastocytosis: Correlation with mast cell burden and implication for defining the category of disease. Int Arch Allergy Immunol 2002;128:136–141.
  22. Austen KF: Systemic mastocytosis. N Engl J Med 1992;326:639–640.
  23. Müller UR, Horat W, Wuthrich B, Conroy M, Reisman RE: Anaphylaxis after hymenoptera stings in three patients with urticaria pigmentosa. J Allergy Clin Immunol 1983;72:685–689.
  24. Kors JW, van Doormaal JJ, de Monchy JG: Anaphylactoid shock following hymenoptera sting as a presenting symptom of systemic mastocytosis. J Intern Med 1993;233:255–258.
  25. Krishna MT, Fearby S, Annila I, Frew A: Hymenoptera stings and serum tryptase. Lancet 2001;357:1527–1528.
  26. Oude Elberink JN, de Monchy JG, Kors JW, van Doormaal JJ, Dubois AE: Fatal anaphylaxis after a yellow jacket sting, despite venom immunotherapy, in two patients with mastocytosis. J Allergy Clin Immunol 1997;99:153–154.
  27. Escribano L, Akin C, Castells M, Orfao A, Metcalfe DD: Mastocytosis: Current concepts in diagnosis and treatment. Ann Hematol 2002;81:677–690.
  28. Valent P, Sperr WR, Schwartz LB, Horny HP: Classification of systemic mast cell disorders: Delineation from immunologic diseases and non mast cell lineage hematopoietic neoplasms. J Allergy Clin Immunol 2004;114:3–11.
  29. Hsu SM, Raine L, Fanger H: Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques: A comparison between ABC and unlabeled antibody (PAP) procedures. J Histochem Cytochem 1981;29:577–580.
  30. Schernthaner GH, Jordan JH, Ghannadan M, Agis H, Bevec D, Nunez R, Escribano L, Majdic O, Willheim M, Worda C, Printz D, Fritsch G, Lechner K, Valent P: Expression, epitope analysis, and functional role of the LFA-2 antigen detectable on neoplastic mast cells. Blood 2001;98:3784–3792.
  31. Hauswirth AW, Sperr WR, Ghannadan M, Schernthaner GH, Jordan JH, Fritsche-Polanz R, Simonitsch-Klupp I, Födinger M, Lechner K, Valent P: A case of smouldering mastocytosis with peripheral blood eosinophilia and lymphadenopathy. Leuk Res 2002;26:601–606.
  32. Butterfield JH, Weiler D, Dewald G, Gleich GJ: Establishment of an immature mast cell line from a patient with mast cell leukemia. Leuk Res 1988;12:345–355.
  33. Schwartz LB: Clinical utility of tryptase levels in systemic mastocytosis and associated hematologic disorders. Leuk Res 2001;25:553–562.
  34. Valent P, Majdic O, Maurer D, Bodger M, Muhm M, Bettelheim P: Further characterization of surface membrane structures expressed on human basophils and mast cells. Int Arch Allergy Appl Immunol 1990;91:198–203.
  35. Sperr WR, Jordan JH, Baghestanian M, Kiener HP, Samorapoompichit P, Semper H, Hauswirth A, Schernthaner GH, Chott A, Natter S, Kraft D, Valenta R, Schwartz LB, Geissler K, Lechner K, Valent P: Expression of mast cell tryptase by myeloblasts in a group of patients with acute myeloid leukemia. Blood 2001;98:2200–2209.
  36. Sperr WR, Stehberger B, Wimazal F, Baghestanian M, Schwartz LB, Kundi M, Semper H, Jordan JH, Chott A, Drach J, Jager U, Geissler K, Greschniok A, Horny HP, Lechner K, Valent P: Serum tryptase measurements in patients with myelodysplastic syndromes. Leuk Lymphoma 2002;43:1097–1105.
  37. Sperr WR, Horny HP, Lechner K, Valent P: Clinical and biologic diversity of leukemias occurring in patients with mastocytosis. Leuk Lymphoma 2000;37:473–486.
  38. Travis WD, Li CY, Hoagland HC, Travis LB, Banks PM: Mast cell leukemia: Report of a case and review of the literature. Mayo Clin Proc 1986;61:957–966.
  39. Valent P, Akin C, Sperr WR, Escribano L, Arock M, Horny HP, Bennett JM, Metcalfe DD: Aggressive systemic mastocytosis and related mast cell disorders: Current treatment options and proposed response criteria. Leuk Res 2003;27:635–641.

Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: July 26, 2004
Accepted: October 11, 2004
Published online: March 16, 2005
Issue release date: March 2005

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 4

ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)

For additional information: https://www.karger.com/IAA


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