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.
| 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 |
| 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 |
| Valent, P; Akin, C; Metcalfe, D D (2007) FIP1L1/PDGFRA is a molecular marker of chronic eosinophilic leukaemia but not for systemic mastocytosis. Eur J Clin Invest 37:153-4 |
| Simons, F Estelle R; Frew, Anthony J; Ansotegui, Ignacio J et al. (2007) Risk assessment in anaphylaxis: current and future approaches. J Allergy Clin Immunol 120:S2-24 |
| Valent, P; Akin, C; Escribano, L et al. (2007) Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 37:435-53 |
| 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 |
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