摘要: 目的：探讨急性髓系白血病相关急性发热性嗜中性皮病的临床特征及诊治。方法：报道1急性髓系白血病相关Sweet综合征分析其临床特点及诊疗经过，复习国内外相关文献。结果：62岁女性患者，因高热1月，皮肤红斑伴破溃半月住院，根据症状、辅助检验、皮肤学活检考虑为急性发热性嗜中性皮病，给予激素治疗后皮疹好转，出现血小板低，行骨髓穿刺见2%的原始细胞，患者拒绝再次骨髓穿刺，血液病诊断无法明确。出院后规律随诊，监测血小板计数均正常，给予激素控制皮疹。7月后，因发热及新发皮疹、四肢无力4天再次入院。因血小板低第二次骨髓穿刺：骨髓中见40.1%的异常髓系原始细胞群，表达MPO，考虑急性髓系白血病，化疗过程中患者很快出现急性重症肺炎(卡氏肺孢子虫感染)，后继发多脏器衰竭死亡。结论：急性髓系白血病相关Sweet综合征患者长期使用激素免疫力低，对肿瘤原发病治疗的耐受性差，预后不佳。应尽早多次骨髓穿刺明确诊断，早期启动原发病的治疗。

Abstract: Objective: To investigate the clinical features and treatment of acute myeloid leukemia-associated acute febrile neutrophilic dermatosis. Methods: One case of acute myeloid leukemia-associated Sweet’s syndrome was reported, and its clinical features and diagnosis and treatment were analyzed, and the relevant domestic and foreign literature was reviewed. Results: A 62-year-old female patient was hospitalized with high fever for one month and erythema with broken skin for half a month. Based on the symptoms, auxiliary tests and dermatological biopsy, she was considered to have acute febrile neutrophilic dermatosis, and her rash improved after glucocorticoid treatment, low platelets were present, 2% of the original cells were found in bone marrow puncture, and the patient refused to repeat bone marrow puncture, so a definitive diagnosis could not be made. The patient was discharged from the hospital with regular follow-ups and normal platelet counts, and was given glucocorticoid to control the rash. 7 months later, she was readmitted to the hospital with fever, new rash, and weakness of the limbs for 4 days. A second bone marrow puncture was performed because of low platelet count: 40.1% abnormal myeloid progenitor cells were found in the bone marrow, expressing MPO, and acute myeloid leukemia was considered, and the patient soon developed acute severe pneumonia (Pneumocystis carinii infection) in the course of chemotherapy, and died of multiple organ failure later on. Conclusion: Patients with Sweet syndrome associated with acute myeloid leukemia have low immunity because of long-term glucocorticoid use, poor tolerance to the treatment of the primary tumor, and poor prognosis. Multiple bone marrow punctures should be performed as early as possible to clarify the diagnosis and initiate the treatment of the primary tumor at an early stage.