Soppi E, Nousiainen T, Seppa A, Lahtinen R
Department of Medicine, University Central Hospital, Kuopio, Finland.
Br J Haematol. 1989 Sep;73(1):43-7. doi: 10.1111/j.1365-2141.1989.tb00217.x.
Three patients with acute febrile neutrophilic dermatosis (AFND, Sweet's syndrome) in association with myelodysplastic syndromes (MDS) are described and, in addition, another 10 published cases are reviewed. In the reviewed patients the male/female ratio is 2:1. The great majority of the patients had decreased or normal leucocyte counts at the time the syndrome developed. Chromosome analysis was available in eight cases: in three the karyotype was normal while five patients had a clonal abnormality. No clinical or laboratory parameters seemed to predict the response to steroids: all but one of the treated patients responded promptly. Moreover, three patients recovered spontaneously. Shortly after the appearance of Sweet's syndrome nine patients developed acute leukaemia and one patient died with hypoplasia. Development of Sweet's syndrome in association with MDS may not be an uncommon clinical finding. The occasional MDS patient developing AFND will often progress into acute leukaemia and thus the occurrence of AFND warrants a bone marrow examination and close follow-up of the patient.
本文描述了3例急性发热性嗜中性皮病（AFND，Sweet综合征）合并骨髓增生异常综合征（MDS）的患者，并对另外10例已发表的病例进行了回顾。在回顾的患者中，男女比例为2:1。绝大多数患者在综合征发生时白细胞计数降低或正常。8例患者进行了染色体分析：3例核型正常，5例患者有克隆性异常。似乎没有临床或实验室参数能够预测对类固醇的反应：除1例接受治疗的患者外，所有患者均迅速产生反应。此外，3例患者自发康复。Sweet综合征出现后不久，9例患者发展为急性白血病，1例患者死于发育不全。Sweet综合征合并MDS可能并非罕见的临床发现。偶尔有MDS患者发生AFND，往往会进展为急性白血病，因此AFND的发生需要进行骨髓检查并对患者进行密切随访。
