TY - JOUR
T1 - Prevention of mast cell activation disorder-associated clinical sequelae of excessive prostaglandin D2 production
AU - Butterfield, Joseph H.
AU - Weiler, Catherine R.
PY - 2008/11
Y1 - 2008/11
N2 - Background: Patients with systemic mastocytosis have increased numbers of mast cells in the bone marrow and other organs, such as the liver, spleen, gastrointestinal tract and skin. Symptoms result from the local and remote effects of mediator release from mast cells and from the local effects of increased mast cell numbers in various organs. Patients with mast cell activation experience many of the same clinical symptoms as do patients with systemic mastocytosis from chronic or spontaneous release of mast cell mediators. We report 4 patients with mast cell activation symptoms from selective release of prostaglandin (PG) D2, but not histamine, and their improvement with aspirin therapy. Methods: Bone marrow biopsy specimens obtained from 4 patients with symptoms suggestive of mastocytosis were examined by tryptase immunostaining. Baseline levels of serum tryptase and urinary 11β-PGF2α and N-methylhistamine were obtained. In 2 of the 4 patients, urinary 11β-PGF2α and N-methylhistamine samples were also measured during acute symptoms. Results: Baseline increase in urinary excretion of the PGD2 metabolite 11β-PGF2α was found in 2 patients. In the remaining 2 patients, baseline levels of urinary 11β-PGF2 α and N-methylhistamine were normal, but during acute symptoms, the excretion of 11β-PGF2α increased markedly. Treatment with aspirin resulted in normalization of 11β-PGF 2α excretion in the 2 patients with elevated baseline levels and in prevention of symptoms in all 4 patients. Conclusions: These results suggest that mast cell activation may be manifested by a selective excessive release of PGD2. These patients respond to administration of aspirin but not to antihistamines.
AB - Background: Patients with systemic mastocytosis have increased numbers of mast cells in the bone marrow and other organs, such as the liver, spleen, gastrointestinal tract and skin. Symptoms result from the local and remote effects of mediator release from mast cells and from the local effects of increased mast cell numbers in various organs. Patients with mast cell activation experience many of the same clinical symptoms as do patients with systemic mastocytosis from chronic or spontaneous release of mast cell mediators. We report 4 patients with mast cell activation symptoms from selective release of prostaglandin (PG) D2, but not histamine, and their improvement with aspirin therapy. Methods: Bone marrow biopsy specimens obtained from 4 patients with symptoms suggestive of mastocytosis were examined by tryptase immunostaining. Baseline levels of serum tryptase and urinary 11β-PGF2α and N-methylhistamine were obtained. In 2 of the 4 patients, urinary 11β-PGF2α and N-methylhistamine samples were also measured during acute symptoms. Results: Baseline increase in urinary excretion of the PGD2 metabolite 11β-PGF2α was found in 2 patients. In the remaining 2 patients, baseline levels of urinary 11β-PGF2 α and N-methylhistamine were normal, but during acute symptoms, the excretion of 11β-PGF2α increased markedly. Treatment with aspirin resulted in normalization of 11β-PGF 2α excretion in the 2 patients with elevated baseline levels and in prevention of symptoms in all 4 patients. Conclusions: These results suggest that mast cell activation may be manifested by a selective excessive release of PGD2. These patients respond to administration of aspirin but not to antihistamines.
KW - Mast cell activation
KW - Mastocytosis
KW - Prostaglandin D
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U2 - 10.1159/000144042
DO - 10.1159/000144042
M3 - Article
C2 - 18622141
AN - SCOPUS:56549084041
SN - 1018-2438
VL - 147
SP - 338
EP - 343
JO - International archives of allergy and immunology
JF - International archives of allergy and immunology
IS - 4
ER -