Lamy T, Dauriac C, Morice P, Le Prisé P Y
Service d'Hématologie Clinique, Hôpital Pontchailou, CHU, Rennes.
Rev Med Interne. 1990 Jul-Aug;11(4):325-8. doi: 10.1016/s0248-8663(05)80868-9.
Four cases of chronic agranulocytosis in patients with systemic diseases different from Felty's syndrome are reported. Two patients had primary Sjögren's syndrome (confirmed in one, suspected in the other), one had systemic lupus erythematosus and the last patient had an unclassifiable connective tissue disease. Only one severe infection was recorded. The pathophysiological mechanisms involved are discussed. There is a strong suspicion that the cytopenia is of immune origin. Corticosteroid therapy was effective in all four patients. One patient who could not tolerate corticosteroids was successfully treated with cyclosporin A.
本文报告了4例患有不同于费尔蒂综合征的全身性疾病的慢性粒细胞缺乏症患者。其中2例患有原发性干燥综合征(1例确诊,1例疑似),1例患有系统性红斑狼疮,最后1例患有无法分类的结缔组织病。仅记录到1例严重感染。文中讨论了所涉及的病理生理机制。强烈怀疑血细胞减少是免疫源性的。皮质类固醇疗法对所有4例患者均有效。1例不能耐受皮质类固醇的患者用环孢素A成功治疗。