Adults with mastocytosis reportedly have poor attention span, irritability, difficulty in concentration, forgetfulness, confusion, mood fluctuation, and depression thought to be due to excess histamine. We have assessed 12 children with mastocytosis to determine if similar behavioral pathology is evident. Results of this study indicate no clear excess of pathology exists in children with mastocytosis. Individual children treated with antihistamines have a nonspecific increase in behavioral difficulties at rates similar to other medically ill groups. No unique behavioral pattern implicating histamine overproduction was identified. We participated in a multi-center consensus conference in 1990 to develop a uniform classification for mastocytosis and uniform evaluation. The results of this conference relied heavily on data obtained at NIH. These consensus recommendations are available and will result in the standard approach to the diagnosis and treatment of this disease. We have completed a study to characterize liver disease in 40 patients with mastocytosis. All patients had routine liver function tests, liver- spleen scan, and liver biopsy, if indicated. We are in the process of reviewing this data to answer the following question: 1. What is the prevalence of liver disease in this population? 2. What are the characteristic features of this liver disease? 3. What are the indications for liver biopsy? 4. Is there a relationship between liver disease and mastocytosis disease category?
| 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 |
| 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 |
| 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 |
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