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Table of Contents
Vol. 127, No. 2, 2002
Issue release date: 2002
Int Arch Allergy Immunol 2002;127:147–152
(DOI:10.1159/000048188)

Mastocytosis: Mediator-Related Signs and Symptoms

Castells M. · Austen K.F.
Brigham and Women’s Hospital, and Harvard Medical School, Boston, Mass., USA

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Abstract

Patients with systemic mastocytosis present symptoms related to the tissue response to the release of mediators from mast cells and to the local mast cell burden. Such patients often have a history of chronic and acute mediator-related symptoms. Most patients have indolent disease with a good prognosis and a normal life span. Symptoms can include pruritus, flushing, syncope, gastric distress, nausea and vomiting, diarrhea, bone pain and neuropsychiatric symptoms, most of which are controlled by medication. Because there is no current cure for mastocytosis, successful therapeutic interventions rely on the recognition of mediator-related symptoms and their treatment, and established intervention approaches for the relatively uncommon leukemic concomitants. Efforts to link a particular mast cell-derived mediator to some aspect of the symptom complex depend on the known actions of the mediator and the efficacy of target-based interventions.



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References

  1. Valent P, Horny HP, Escribano L, Longley BJ, Chin YL, 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.
  2. Horan RF, Austen KF: Systemic mastocytosis: Retrospective review of a decade’s clinical experience at the Brigham and Women’s Hospital. J Invest Dermatol 1991;96:5S–14S.
  3. Muller 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.

    External Resources

  4. 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.
  5. Soter NA: Mastocytosis and the skin. Hematol Oncol Clin North Am 2000;14:537–555.

    External Resources

  6. Hannafort R, Rogers M: Presentation of cutaneous mastocytosis in 173 children. Australas J Dermatol 2001;42:15–21.

    External Resources

  7. Murphy M, Walsh D, Drumm B, Watson R: Bullous mastocytosis, a fatal outcome. Pediatr Dermatol 1999;16:452–455.
  8. Ludatscher RM, Haim S, Gellei B, Cohen A: Comparative ultrastructure study of mast cells in mastocytoma and mastocytosis. Dermatologica 1977;155:80.

    External Resources

  9. Wiedner N, Horan RF, Austen KF: Mast cell phenotypes in indolent forms of mastocytosis. Am J Pathol 1992;140:847–857.
  10. Tharp MD, Chaker B, Glass MJ, Burton R, Seelig LL Jr: In vitro functional reactivities of cutaneous mast cells from patients with mastocytosis. J Invest Dermatol 1987;89:264.

    External Resources

  11. Gurish MF, Hong T, Abonia JP, Arya A, Friend DS, Parker CM, Austen KF: Intestinal mast cell progenitors require CD49dβ7 (a4β7 integrin) for tissue-specific homing. J Exp Med 2001;194:1–11.
  12. Longley BJ Jr, Morganroth GS, Tyrrell L, Ding TG, Anderson DM, Williams DE, Halaban R: Altered metabolism of mast-cell growth factor (c-kit ligand) in cutaneous mastocytosis. N Engl J Med 1993;328:1302–1307.
  13. Castells MC, Friend DS, Bunnell CA, Hu X, Kraus M, Osteen RT, Austen KF: The presence of membrane-bound stem cell factor on highly immature nonmetachromatic mast cells in the peripheral blood of a patient with aggressive systemic mastocytosis. J Allergy Clin Immunol 1996;98:831–840.

    External Resources

  14. Metcalfe DD, Akin C: Mastocytosis: Molecular mechanisms and clinical disease heterogeneity. Leuk Res 2000;25:577–582.

    External Resources

  15. Dror Y, Leaker M, Caruana G, Bernstein A, Freedman MH: Mastocytosis cells bearing a c-kit activating point mutation are characterized by hypersensitivity to stem cell factor and increased apoptosis. Br J Haematol 2000;108:729–736.
  16. Soter NA, Lewis RA, Corey EJ, Austen KF: Local effects of synthetic leukotrienes (LTC4, LTD4, LTE4 and LTB4) in human skin. J Invest Dermatol 1983;80:115–119.
  17. Jensen RT: Gastrointestinal abnormalities and involvement in systemic mastocytosis. Hematol Oncol Clin North Am 2000;14:579–623.
  18. Cherner JA, Jensen RT, Dubois A, O’Dorisio TM, Gardner JD, Metcalfe DD: Gastrointestinal dysfunction in systemic mastocytosis. Gastroenterology 1988;95:657–667.

    External Resources

  19. Johnson AC, Johnson S, Lester PD, Halter S, James E: Systemic mastocytosis and mastocytosis-like syndrome: Radiologic features of gastrointestinal manifestations. South Med J 1988;81:729–733.

    External Resources

  20. Libel R, Biddle WL, Miner PB Jr: Evaluation of anorectal physiology in patients with increased mast cells. Dig Dis Sci 1993;38:877–881.
  21. Soter NA, Austen KF, Wasserman SI: Oral disodium cromoglycate in the treatment of systemic mastocytosis. N Engl J Med 1979;301:465–469.
  22. Mican JM, DiBisceglie AM, Fong TL, Travis WD, Kleiner DE, Baker B, Metcalfe DD: Hepatic involvement in mastocytosis: Clinicopathologic correlations in 41 cases. Hepatology 1995;22:1163–1170.

    External Resources

  23. Horny HP, Kaiserling E, Campbell M, Paswaresh MR, Lennert K: Liver findings in generalized mastocytosis. Cancer 1989;63:532–538.

    External Resources

  24. Sperr WR, Escribano L, Jordan JH, Schernthaner GH, Kundi M, Horny HP, Valent P: Morphologic properties of neoplastic mast cell: Delineation of stages of maturation and implication for cytological grading of mastocytosis. Leuk Res 2001;25:529–536.
  25. Horny HP, Ruck MT, Kaiserling E: Spleen findings in generalized mastocytosis. A clinicopathologic study. Cancer 1992;70:459–468.
  26. Mekori YA: Lymphoid tissues and the immune system in mastocytosis. Hematol Oncol Clin North Am 2000;14:569–577.

    External Resources

  27. Duffy TP: Getting the story right. N Engl J Med 1993;328:1333.
  28. Patella V, Marino I, Lamparter B, Arbustini E, Adt M, Marone G: Human heart mast cells. J Immunol 1995;154:2855.
  29. Frangogiannis NS, et al: Resident cardiac mast cells degranulate and release preformed TNFα, initiating the cytokine cascade in experimental canine myocardial ischemia/reperfusion. Circulation 1998;98:699.
  30. Roberts LJ, Sweetman BJ, Lewis RA, Austen KF, Oates JA: Increased production of PGD2 in patients with systemic mastocytosis. N Engl J Med 1980;303:1400.

    External Resources

  31. Rogers MP, Bloomingdale K, Murawski BJ, Soter NA, Reich P, Austen KF: Mixed organic brain syndrome as a manifestation of systemic mastocytosis. Psychosom Med 1986;48:437–447.
  32. Gerashchenko D, Okano Y, Urade Y, Inoue S, Hayaishi O: Strong rebound of wakefulness follows PGD2 or adenosine A2a receptor agonist-induced sleep. J Sleep Res 2000;9:81–87.
  33. Pinzar E, Kanaoka Y, Inui T, Eguchi N, Urade Y, Hayaishi O: Prostaglandin D synthase gene is involved in the regulation of non-rapid eye movement sleep. Proc Natl Acad Sci USA 2000;97:4903–4907.
  34. Horan RF, Sheffer AL, Austen KF: Cromolyn sodium in the management of systemic mastocytosis. J Allergy Clin Immunol 1990;85:852.
  35. Scmidt M, Dercken C, Loke O, Reimann S, Diederich S, Blasius S, Heidenraich S: Pulmonary manifestations of systemic mast cell disease. Eur Respir J 2000;15:623–625.
  36. Worobec AS, Akin C, Scott LM, Metcalfe DD: Mastocytosis complicating pregnancy. Obstet Gynecol 2000;95:391–395.


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