Grüne S, Panizzon R, Egli F, Siegenthaler W, Greminger P
Department für Innere Medizin, Universitätsspital Zürich.
Dtsch Med Wochenschr. 1996 Jul 26;121(30):939-42. doi: 10.1055/s-2008-1043091.
Two months after being diagnosed as having refractory anaemia with an excess of blasts in transformation (RAEB-T), a 62-year-old man presented in the emergency room with fever (40 degrees C) for two weeks and scattered deep-red macular indolent efflorescences over the chest, back, face and thighs. Other than splenomegaly there were no significant findings on physical examination.
Erythrocyte sedimentation rate was increased to 38 mm in the first hour. Haemoglobin concentration and platelet count were at the lower limits of normal, white cell count within the normal range. Differential count: 60 erythroblasts per 100 leukocytes and 33.5 blast cells. Two skin biopsies revealed massive oedema in the upper corium and focal erythrocyte extravasations. There were perivascular and perifollicular inflammatory infiltrates in the deeper layers and elastosis of the corium. There was no leukocytoclastic vasculitis. These findings established the diagnosis of Sweet syndrome and erythroleukaemia.
The erythroleukaemia was treated symptomatically and the skin changes gradually receded under prednisone, 1 mg/kg, but new spots occurred when the prednisone dose was halved. Candida oesophagitis occurred as a complication of the erythroleukaemia. Chest radiogram showed diffuse infiltrates in both upper lobes of the lung. Despite intensive antimycotic and antibiotic treatment the patient died 10 days later from pulmonary aspergillosis.
This case report describes the rare occurrence of Sweet syndrome during the transformation from a myelodysplastic Syndrome to erythroleukaemia.
一名62岁男性在被诊断为伴有原始细胞过多的难治性贫血（RAEB-T）两个月后，因发热（40摄氏度）两周且胸部、背部、面部和大腿出现散在的深红色黄斑性无痛性皮疹而就诊于急诊室。体格检查除脾肿大外无其他明显发现。
红细胞沉降率在第一小时升至38毫米。血红蛋白浓度和血小板计数处于正常下限，白细胞计数在正常范围内。分类计数：每100个白细胞中有60个成红细胞和33.5个原始细胞。两次皮肤活检显示真皮上层有大量水肿和局灶性红细胞外渗。真皮深层有血管周围和毛囊周围炎性浸润以及真皮弹性组织变性。无白细胞破碎性血管炎。这些发现确立了Sweet综合征和红白血病的诊断。
红白血病进行了对症治疗，皮肤改变在1毫克/千克泼尼松治疗下逐渐消退，但泼尼松剂量减半时出现了新的皮疹。念珠菌性食管炎作为红白血病的并发症出现。胸部X线片显示两肺上叶有弥漫性浸润。尽管进行了强化抗真菌和抗生素治疗，患者10天后死于肺曲霉菌病。
本病例报告描述了在骨髓增生异常综合征向红白血病转化过程中罕见的Sweet综合征发生情况。
