Ababou Mounir, El Maaroufi Hicham, Hammani Adnane, Jennane Selim, Mahtat El Mehdi, Mikdame Mohammed, Doghmi Kamal
Department of Clinical Hematology, Military Hospital Mohammed V, Mohammed V University, Rabat, Morocco.
Faculty of Medicine and Pharmacy of Rabat, Mohammed V University, Rabat, Morocco.
Case Rep Hematol. 2020 Sep 9;2020:8818270. doi: 10.1155/2020/8818270. eCollection 2020.
Acute laryngeal dyspnea is a life-threatening emergency, and the causes in adults are most often laryngeal tumors or inflammatory edema. Lymphoma of the larynx and especially the mantle cell type is extremely rare. . We report a case of a 43-year-old woman with no particular pathological history. She presented with progressive dyspnea which has evolved towards an inspiratory bradypnea that worsened until she ultimately required an emergency tracheotomy. Biopsies revealed mantle cell lymphoma. The patient has been staged IVB MIPI 6, and she was treated by immunochemotherapy followed by ASCT. The therapeutic evaluation shows a complete remission, 18 months after, and the patient was always disease free.
The laryngeal localization of the mantle cell lymphoma is extremely rare; it may present catastrophically with acute airway obstruction. The diagnosis is mostly histological, hence the interest of deep biopsy. Given its rarity, the therapeutic strategy must be discussed case by case in a multidisciplinary consultation meeting.
急性喉呼吸困难是一种危及生命的急症,成人病因多为喉部肿瘤或炎性水肿。喉淋巴瘤尤其是套细胞型极为罕见。我们报告一例43岁无特殊病史的女性患者。她表现为进行性呼吸困难,逐渐发展为吸气性呼吸过缓,病情不断恶化,最终需要紧急气管切开术。活检显示为套细胞淋巴瘤。该患者分期为IVB期,MIPI评分为6分,接受了免疫化疗,随后进行了自体造血干细胞移植(ASCT)。治疗评估显示18个月后完全缓解,患者一直无病生存。
套细胞淋巴瘤的喉部定位极为罕见;它可能以急性气道梗阻的灾难性形式出现。诊断主要依靠组织学检查,因此深部活检很有必要。鉴于其罕见性,治疗策略必须在多学科会诊会议中逐例讨论。