弥漫性皮肤肥大细胞增多症中血小板活化因子（PAF）及血小板聚集内源性抑制剂的出现情况。
Occurrence of platelet-activating factor (PAF) and an endogenous inhibitor of platelet aggregation in diffuse cutaneous mastocytosis.
作者信息
Macpherson J L, Kemp A, Rogers M, Mallet A I, Toia R F, Spur B, Earl J W, Chesterman C N, Krilis S A
机构信息
University of New South Wales School of Medicine, St. George Hospital, Sydney, Australia.
出版信息
Clin Exp Immunol. 1989 Sep;77(3):391-6.
We have identified PAF in the blister fluid from a patient with bullous mastocytosis, a rare form of mast-cell disease. We have found a novel endogenous inhibitor of platelet aggregation which obscured the presence of the PAF in unprocessed blister fluid and in ethanol or lipid extracts. The PAF was characterized by the demonstration of chromatographic, mass spectral and biological properties identical to those of authentic PAF. Thus this is the first demonstration of PAF in biological fluid from a patient with mastocytosis. High levels of immunoreactive prostaglandin D2 (PGD2) and histamine were also present in the blister fluid. The interaction between PAF and the inhibitor of platelet aggregation in patients with systemic mastocytosis may provide an explanation for some of the manifestations of the disease, in particular the episodic hypotension, cutaneous flushing and pallor.
我们已在大疱性肥大细胞增多症（一种罕见的肥大细胞疾病）患者的水疱液中鉴定出了血小板活化因子（PAF）。我们发现了一种新型的血小板聚集内源性抑制剂，它掩盖了未处理的水疱液以及乙醇或脂质提取物中PAF的存在。通过证明其色谱、质谱和生物学特性与 authentic PAF相同，对PAF进行了表征。因此，这是首次在肥大细胞增多症患者的生物体液中证实存在PAF。水疱液中还存在高水平的免疫反应性前列腺素D2（PGD2）和组胺。系统性肥大细胞增多症患者中PAF与血小板聚集抑制剂之间的相互作用可能为该疾病的某些表现，特别是发作性低血压、皮肤潮红和苍白，提供一种解释。
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