Rafii M, Firooznia H, Golimbu C, Balthazar E
Clin Orthop Relat Res. 1983 Nov(180):260-7.
Systemic mastocytosis is a multiorgan disease that most commonly affects the skin and skeletal system. Radiographically, the skeletal changes in the majority of patients consist of either a wide-spread mixture of bone lysis and osteosclerosis or generalized osteoporosis. The osteoporotic form is less well known but may lead to severe generalized demineralization and pathologic fractures. Mast cells secrete a number of substances, two of which (heparin and prostaglandins) are believed to have a role in the induction of osteoporosis. Sclerotic lesions are induced by another mast cell by-product, histamine. One hundred seventy-eight cases of bony mastocytosis have been reported in the literature, including the four patients in the present report. Special staining procedures are necessary for identification of mast cells. Diagnosis may be delayed in patients who do not have the skin lesions (urticaria pigmentosa) and in the osteoporotic form of the disease.
系统性肥大细胞增多症是一种多器官疾病，最常累及皮肤和骨骼系统。影像学上，大多数患者的骨骼改变包括广泛的骨溶解和骨硬化混合或全身性骨质疏松。骨质疏松形式较不为人所知，但可能导致严重的全身性脱矿质和病理性骨折。肥大细胞分泌多种物质，其中两种（肝素和前列腺素）被认为在骨质疏松的诱导中起作用。硬化性病变由另一种肥大细胞副产物组胺诱导。文献中已报道178例骨肥大细胞增多症病例，包括本报告中的4例患者。识别肥大细胞需要特殊的染色程序。对于没有皮肤病变（色素性荨麻疹）的患者以及疾病的骨质疏松形式，诊断可能会延迟。
