Lortholary O, Casassus P, Laroche L, Lortholary P, Diebold J
Département de Médecine Interne, Hôpital Avicenne, Bobigny.
Presse Med. 1990 Jan 27;19(3):125-8.
Systemic mastocytosis is a rare, often undiagnosed disease characterized by the proliferation of mast cells in several tissues. The clinical symptoms are related to the mast cell infiltrates, but also to the release of numerous mediators. Malignant mastocytosis is a term that refers to two different entities: aggressive mastocytosis, where the outcome with a myeloproliferative disease, where the latter governs the prognosis. The potential severity of these diseases justifies the development of more intensive therapeutic measures.
系统性肥大细胞增多症是一种罕见的、常常未被诊断的疾病,其特征是肥大细胞在多个组织中增殖。临床症状与肥大细胞浸润有关,但也与多种介质的释放有关。恶性肥大细胞增多症是一个术语,指两种不同的实体:侵袭性肥大细胞增多症,其预后与骨髓增殖性疾病相关,后者决定预后。这些疾病的潜在严重性证明了更强化治疗措施的必要性。