Brumann Mareen, Bogner Viktoria, Völkl Andreas, Sotlar Karl, Euler Ekkehard, Mutschler Wolf
Department of Trauma Surgery, University Hospital Munich, Ludwig-Maximilians-University, Nussbaumstr. 20, Munich 80336, Germany.
Department of Hematology and Oncology, University Hospital Munich, Ludwig-Maximilians-University, Ziemsenstr. 1, Munich 80336, Germany.
Patient Saf Surg. 2014 Jun 26;8:28. doi: 10.1186/1754-9493-8-28. eCollection 2014.
Necrotizing fasciitis is characterized by a fulminant destruction of the soft tissue with an alarmingly high mortality rate. One of the main reasons for the continued high mortality is due to the challenge to punctual recognize and diagnose this disease, as specific cutaneous signs can vary or even be missing early in its evolution - especially in case of simultaneous first manifestation of an acute leukemia.
An untypical case of necrotizing fasciitis disease in a young patient with the first diagnosis of acute myeloid leukemia is presented. After her induction chemotherapy the only presenting clinical sign was fever in the presence of severe neutropenia without an evident infectious focus. After a few days a painless confluent, erythematous, pustular skin rash with a central necrosis on lateral thigh appeared. Escherichia coli was isolated from blood cultures. Surgical debridement was performed and showed subcutaneous tissue, fascia and underlying muscle around the site of initial cutaneous manifestation with typical necrosis on exploration. But, initially taken skin biopsy did not show any typical histopathological findings like bacteria or inflammatory cells confirming necrotizing fasciitis. Nevertheless, the intraoperative findings were impressive and highly indicative for a necrotizing soft tissue infection, so that the patient was treated according to clinical guidelines with extensive recurrent surgical debridement, broad-spectrum antibiotics and intensive care therapy. After recovering from NF, she successfully underwent further chemotherapy and stem cell transplantation.
The presented case highlights the risk of potential misinterpretation, delayed diagnosis and treatment of necrotizing fasciitis in patients presenting with an untypical clinical and histopathological manifestation of necrotizing fasciitis as a result of severe neutropenia following chemotherapy for acute myeloid leukemia.
坏死性筋膜炎的特征是软组织迅速破坏,死亡率高得惊人。持续高死亡率的主要原因之一是及时识别和诊断这种疾病存在挑战,因为特定的皮肤体征在疾病早期可能会有所不同,甚至缺失——尤其是在急性白血病同时首次出现的情况下。
本文介绍了一名初诊为急性髓系白血病的年轻患者发生的坏死性筋膜炎非典型病例。在她进行诱导化疗后,唯一出现的临床症状是在严重中性粒细胞减少的情况下发热,且无明显感染灶。几天后,患者大腿外侧出现无痛性融合性红斑脓疱性皮疹,中央坏死。血培养分离出大肠杆菌。进行了手术清创,术中发现最初皮肤表现部位周围的皮下组织、筋膜和深层肌肉有典型坏死。但是,最初进行的皮肤活检未显示任何典型的组织病理学发现,如证实坏死性筋膜炎的细菌或炎性细胞。尽管如此,术中发现令人印象深刻,高度提示坏死性软组织感染,因此根据临床指南对患者进行了广泛的反复手术清创、广谱抗生素治疗和重症监护治疗。从坏死性筋膜炎康复后,她成功接受了进一步的化疗和干细胞移植。
本病例突出了因急性髓系白血病化疗后严重中性粒细胞减少导致坏死性筋膜炎出现非典型临床和组织病理学表现的患者中存在潜在误诊、延迟诊断和治疗的风险。