Local radiotherapy was successful in decreasing refractory bone pain in mastocytosis patients with significant bone erosion. This severe intractable bone pain is seen in patients with aggressive disease and is usually treated unsuccessfully with narcotic analgesics. Studies are underway to assess cytokine profiles in mastocytosis patients. Suction blisters are raised over urticaria pigmentosa lesions which contain collections of mast cells. Blister fluid is assayed for cytokines appearing in blister fluid over several hours. A new improved serum assay for mast cell-derived tryptase may result in improved diagnosis for systemic mast cell disorders. Significant liver disease is seen in association with aggressive mast cell disease (Categories II and III). Pathology includes mast cell infiltrates, inflammation around the portal triads, and fibrosis. A retrospective review of the bone scans of 75 mastocytosis patients has demonstrated that bone scanning provides an efficient, noninvasive method of assessing and following active bone and bone marrow involvement. As the disease progresses, abnormalities on bone scan become increasingly diffuse. Progression of disease on serial bone scans occurs in patients more likely to have a malignant course.

Agency
National Institute of Health (NIH)
Institute
National Institute of Allergy and Infectious Diseases (NIAID)
Type
Intramural Research (Z01)
Project #
1Z01AI000249-12
Application #
3768757
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
12
Fiscal Year
1993
Total Cost
Indirect Cost
City
State
Country
United States
Zip Code
Akin, Cem; Valent, Peter; Metcalfe, Dean D (2010) Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol 126:1099-104.e4
Kulka, Marianna; Metcalfe, Dean D (2010) Isolation of tissue mast cells. Curr Protoc Immunol Chapter 7:Unit 7.25
Lahortiga, Idoya; Akin, Cem; Cools, Jan et al. (2008) Activity of imatinib in systemic mastocytosis with chronic basophilic leukemia and a PRKG2-PDGFRB fusion. Haematologica 93:49-56
Simons, F E R; Frew, A J; Ansotegui, I J et al. (2008) Practical allergy (PRACTALL) report: risk assessment in anaphylaxis. Allergy 63:35-7
Peavy, Richard D; Metcalfe, Dean D (2008) Understanding the mechanisms of anaphylaxis. Curr Opin Allergy Clin Immunol 8:310-5
Jensen, Bettina M; Metcalfe, Dean D; Gilfillan, Alasdair M (2007) Targeting kit activation: a potential therapeutic approach in the treatment of allergic inflammation. Inflamm Allergy Drug Targets 6:57-62
Carter, Melody C; Robyn, Jamie A; Bressler, Peter B et al. (2007) Omalizumab for the treatment of unprovoked anaphylaxis in patients with systemic mastocytosis. J Allergy Clin Immunol 119:1550-1
Akin, Cem; Scott, Linda M; Kocabas, Can N et al. (2007) Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with ""idiopathic"" anaphylaxis. Blood 110:2331-3
Akin, Cem; Soto, Darya; Brittain, Erica et al. (2007) Tryptase haplotype in mastocytosis: relationship to disease variant and diagnostic utility of total tryptase levels. Clin Immunol 123:268-71
Maric, Irina; Robyn, Jamie; Metcalfe, Dean D et al. (2007) KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities. J Allergy Clin Immunol 120:680-7

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