Butterfield Joseph H, Weiler Catherine R
Division of Allergic Diseases, Mayo Clinic, Rochester, Minn. 55905, USA.
Int Arch Allergy Immunol. 2008;147(4):338-43. doi: 10.1159/000144042. Epub 2008 Jul 12.
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) D(2), but not histamine, and their improvement with aspirin therapy.
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 11beta-PGF(2)(alpha) and N-methylhistamine were obtained. In 2 of the 4 patients, urinary 11beta-PGF(2)(alpha) and N-methylhistamine samples were also measured during acute symptoms.
Baseline increase in urinary excretion of the PGD(2) metabolite 11beta-PGF(2)(alpha) was found in 2 patients. In the remaining 2 patients, baseline levels of urinary 11beta-PGF(2)(alpha) and N-methylhistamine were normal, but during acute symptoms, the excretion of 11beta-PGF(2)(alpha) increased markedly. Treatment with aspirin resulted in normalization of 11beta-PGF(2)(alpha) excretion in the 2 patients with elevated baseline levels and in prevention of symptoms in all 4 patients.
These results suggest that mast cell activation may be manifested by a selective excessive release of PGD(2). These patients respond to administration of aspirin but not to antihistamines.
系统性肥大细胞增多症患者的骨髓及其他器官（如肝脏、脾脏、胃肠道和皮肤）中的肥大细胞数量增加。症状源于肥大细胞释放介质的局部和远处效应以及各个器官中肥大细胞数量增加的局部效应。肥大细胞活化的患者会出现许多与系统性肥大细胞增多症患者相同的临床症状，这些症状是由肥大细胞介质的慢性或自发释放引起的。我们报告了4例因前列腺素（PG）D2选择性释放而非组胺释放而出现肥大细胞活化症状的患者，以及他们使用阿司匹林治疗后的病情改善情况。
对4例有肥大细胞增多症症状提示的患者进行骨髓活检标本的类胰蛋白酶免疫染色检查。获取血清类胰蛋白酶、尿11β - PGF2α和N - 甲基组胺的基线水平。在4例患者中的2例，急性症状期间还检测了尿11β - PGF2α和N - 甲基组胺样本。
2例患者的PGD2代谢产物11β - PGF2α的尿排泄基线增加。其余2例患者尿11β - PGF2α和N - 甲基组胺的基线水平正常，但在急性症状期间，11β - PGF2α的排泄显著增加。阿司匹林治疗使2例基线水平升高的患者的11β - PGF2α排泄恢复正常，并预防了所有4例患者的症状。
这些结果表明，肥大细胞活化可能表现为PGD2的选择性过度释放。这些患者对阿司匹林治疗有反应，但对抗组胺药无反应。
