Patients with mastocytosis are more likely to develop significant liver pathology if they have category II disease (mastocytosis with an associated hematologic disorder) or category III disease aggressive mastocytosis). Liver pathology includes mast cell hyperplasia and fibrosis, sometimes leading to portal hypertension. Patients with category I disease (indolent mastocytosis) are less likely to develop these problems. Liver-spleen scans were of value in assessing liver size, and in identifying other types of problems such as liver hemangiomas. Based on these findings routine liver biopsy is not recommended in the evaluation of mastocytosis. Liver biopsy should only be performed on patients with laboratory and scan evidence of significant liver disease. Preliminary studies are underway to assess cytokine profiles in mastocytosis patients, with emphasis on stem cell factor. 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. It is hoped that this study will identify an abnormal cytokine profile associated with mast cell hyperplasia.
| 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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