Asymptomatic Systemic Mastocytosis in an Elderly Female

Systemic Mastocytosis: An Intriguing Disorder

An elusive case of systemic mastocytosis

A 40 year old woman with recurrent “idiopathic” anaphylaxis was referred. Past history revealed a right ovarian mucinous cyst adenoma that was surgically removed. Medications included celebrex and alesse. Six months following the oopherectomy at age 15, she began to experience anaphylaxis every 2 months. Typically these occurred in late afternoon, not on an empty stomach, and half related to ph...

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.

Gastrointestinal involvement in systemic mastocytosis.

Four consecutive patients with systemic mastocytosis were studied. One patient had a malabsorption syndrome with only minor histological changes of the intestinal mucosa. Another patient with ulcer diathesis had a gastric secretory pattern resembling Zollinger-Ellison syndrome. Serum gastrin and histamine levels were consistently normal in all patients. Endoscopy of stomach and colon disclosed ...

Indolent systemic mastocytosis.

circular macules, less than 1 cm in diameter, located on his trunk and extremities [Figure A]. Wheals and surrounding erythema developed in the lesions after rubbing (positive Darier sign). Laboratory analyses showed mild eosinophilia with an increased total tryptase level of 40 ng/ml. Densitometry was normal. With the strong clinical suspicion of cutaneous mastocytosis, a skin biopsy was perfo...