1/7. mastocytosis associated with severe wasp sting anaphylaxis detected by elevated serum mast cell tryptase levels.A markedly elevated serum level of mast cell tryptase (77.6 microg/L; 95th percentile in normals 13.5 microg/L) was detected in a patient treated for 5 years with wasp venom immunotherapy because of severe anaphylaxis following a wasp sting. Retrospective analysis of stored serum samples taken during the course of immunotherapy revealed that the tryptase level had been elevated for at least 3 years. Despite several dermatological examinations, skin changes of mastocytosis had been missed. Re-examination of the patient revealed sparse macules on the thorax and thighs; Darier's sign was negative. Histologically, mast cell accumulation in these lesions was demonstrable. No signs of systemic mastocytosis were detected. The most appropriate diagnosis was telangiectasia macularis eruptiva perstans. Even in patients with highly elevated tryptase levels, mastocytosis may go undiagnosed. As mastocytosis predisposes to severe anaphylaxis, the condition should be looked for in patients with such reactions by clinical examination and measurement of serum tryptase levels.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/7. Dental management of a pediatric patient with mastocytosis: a case report.mastocytosis is a heterogeneous group of clinical disorders characterized by an excessive number of normal mast cells in a variety of tissues (skin, bone marrow, liver, spleen and lymph nodes). It is most often seen in the skin in pediatric-onset mastocytosis presenting as urticaria pigmentosa. Children with this disorder are on a strict avoidance protocol of triggering factors to decrease the likelihood of life-threatening anaphylactic reactions. Close monitoring and the avoidance of known histamine-releasing drugs is necessary in the pediatric dental office, as is a readiness to use resuscitative measures. A case of a 4-year, 6-month-old pediatric dental patient with mastocytosis is presented. Dental treatment was provided in an ambulatory setting utilizing nitrous oxide, oxygen analgesia and H1 and H2 antihistamines to prevent mast cell degranulation and to provide sedation.- - - - - - - - - - ranking = 1.5keywords = mastocytosis (Clic here for more details about this article) |
3/7. anaphylaxis to insect sting associated with urticaria pigmentosa.anaphylaxis associated with insect stings has been reported to cause approximately 40 deaths per year in the united states. immunotherapy with venom extracts is a well-established method of treatment of allergy to insect stings. The duration of therapy is based mainly on the initial symptoms and the presence or absence of systemic symptoms during therapy. Evaluation of immunoglobulin e and immunoglobulin g levels as well as repeat skin tests and sting challenges may also provide some additional benefit but are not as useful as the former two criteria. patients with mastocytosis have a particularly increased risk for anaphylaxis after insect stings. There are many case reports of individuals first diagnosed with mastocytosis after an episode of anaphylaxis after an insect sting, in addition these patients tend to have more severe reactions as well as repeated episodes of systemic reactions during immunotherapy. early diagnosis of mastocytosis and proper treatment can contribute greatly to the outcome in patients who present with venom allergy.- - - - - - - - - - ranking = 0.75keywords = mastocytosis (Clic here for more details about this article) |
4/7. Indolent systemic mastocytosis with elevated serum tryptase, absence of skin lesions, and recurrent severe anaphylactoid episodes.BACKGROUND: In contrast to aggressive mastocytosis, patients with indolent systemic mastocytosis (ISM) usually present with urticaria pigmentosa-like skin lesions. In those who lack skin lesions, mastocytosis is often overlooked or confused with endocrinologic, allergic, or other internal disorders. CASE REPORT AND RESULTS: We report on a 33-year-old male patient in whom severe hypotensive episodes occurred after contact with ants or yellow jackets. Since no specific IgE was detected, the serum tryptase concentration was measured and found to be clearly elevated (70 ng/ml). Consecutive staging and examination of the bone marrow revealed ISM. The patient was advised to circumvent insect contact, to take antihistamines on demand, and to carry an epinephrine self-injector for emergency events. In a retrospective analysis of 40 patients seen between 1988 and 2003, only 2 had a life-threatening mediator-related episode before ISM was diagnosed. CONCLUSIONS: Our report confirms the diagnostic value of tryptase in patients with suspected mastocytosis. In addition, the report suggests that the lack of typical skin lesions does not exclude an indolent form of mastocytosis even if the serum tryptase is clearly elevated. Finally, our case further shows that mastocytosis can be an important differential diagnosis to be considered in patients with unexplained anaphylactoid or other mediator-related symptoms.- - - - - - - - - - ranking = 2.5keywords = mastocytosis (Clic here for more details about this article) |
5/7. Recurrent syncope and anaphylaxis as presentation of systemic mastocytosis in a pediatric patient: case report and literature review.mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbers of mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping, syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa, which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.- - - - - - - - - - ranking = 1.5keywords = mastocytosis (Clic here for more details about this article) |
6/7. Fatal anaphylaxis in systemic mastocytosis.A 42-year-old woman died after an episode of anaphylaxis associated with a raised serum histamine level. A diagnosis of systemic mastocytosis was established, with lymphadenopathy and hepatosplenomegaly, not associated with the usually pre-existing skin lesions of urticaria pigmentosa.- - - - - - - - - - ranking = 1.25keywords = mastocytosis (Clic here for more details about this article) |
7/7. anaphylaxis after hymenoptera stings in three patients with urticaria pigmentosa.Three patients with urticaria pigmentosa are reported who developed symptoms of anaphylaxis after hymenoptera stings. serum IgE antibodies to various hymenoptera venoms could not be detected in any of the patients. skin tests were completely negative in one patient, and borderline reactions with honeybee and yellow jacket venom, respectively, were found in the other two. Peripheral blood leukocytes of these latter two patients did not release significant amounts of histamine after exposure to the respective venoms. In patients with mastocytosis, anaphylaxis after insect stings may not be IgE-mediated but due to mediator release by the pharmacologic action of histamine liberators normally present in hymenoptera venoms.- - - - - - - - - - ranking = 0.25keywords = mastocytosis (Clic here for more details about this article) |