髓样肉瘤 (MS) 是一种罕见的髓外肿瘤肿块，具有进展为急性髓系白血病 (AML) 的高风险，MS 患者通常接受 AML 方案治疗。然而，由于缺乏临床特异性，多发性硬化症经常被误诊。携带肿瘤蛋白 p53 (TP53) 突变和复杂核型的多发性硬化症患者被认为预后较差。本研究报告了一例伴有 TP53 (V173G) 相关骨髓增生异常综合征 (MDS) 的淋巴结 MS 病例。该肿块首先被认为是淋巴瘤并按此治疗。然而，免疫组织化学分析显示细胞 CD43、髓过氧化物酶和 CD117 呈阳性，该患者后来被诊断为 MS 合并 MDS。患者在第一个周期的化疗后进入完全缓解，并在第二个周期的化疗后出现血小板、红细胞和白细胞计数减少。第三次化疗后，出现粒细胞缺乏症，导致难治性肺炎，最终因呼吸衰竭而死亡。 TP53相关MDS的MS发病率低，预后差，生存期短。 MS的临床表现缺乏特异性，容易误诊，导致延误诊断和治疗，最终恶化患者的预后。因此，对于淋巴结肿大的患者，应尽早进行淋巴结活检，早期治疗，以延长生存期。版权所有：©2024 毛邓。

Myeloid sarcoma (MS) is a rare extramedullary tumor mass that carries a high risk of progression to acute myeloid leukemia (AML), and patients with MS are commonly treated with the AML regimen. However, MS is frequently misdiagnosed due to its lack of clinical specificity. Patients with MS who harbor tumor protein p53 (TP53) mutations and complex karyotypes are considered to have a poorer prognosis. The present study reports a case of lymph node MS with TP53 (V173G)-related myelodysplastic syndrome (MDS). The mass was first considered to be a lymphoma and treated as such. However, following immunohistochemical analysis, which revealed cells positive for CD43, myeloperoxidase and CD117, the patient was later diagnosed with MS combined with MDS. The patient went into complete remission after the first cycle of chemotherapy, and showed a decrease in platelet, red blood cell and white blood cell counts following the second cycle of chemotherapy. After the third chemotherapy, agranulocytosis occurred, leading to refractory pneumonia and eventually death due to respiratory failure. MS with TP53-related MDS has a low incidence rate, a poor prognosis and a short survival time. The clinical manifestations of MS are non-specific and easy to misdiagnose, leading to delayed diagnosis and treatment, and ultimately worsening the prognosis of the patients. Therefore, a lymph node biopsy should be performed as soon as possible for patients with lymph node enlargement, and early treatment should be carried out to prolong the survival period.Copyright: © 2024 Mao and Deng.