Vignes S, Brasnu D, Hénégar C, Tiev K P, Généreau T, Cabane J
Service de médecine interne, hôpital Saint-Antoine, 184, rue du Faubourg-Saint-Antoine, 75571 Paris, France.
Rev Med Interne. 2000 Dec;21(12):1121-5. doi: 10.1016/s0248-8663(00)00272-1.
The larynx is a rare site of deposition for amyloidosis. Diagnosis may be delayed and evoked in patients with prolonged hoarseness. We have reported two cases of laryngeal amyloidosis.
One man and one woman suffered from hoarseness during one and three years, respectively. Laryngoscopic examination showed diffuse infiltration of the larynx. Amyloidosis was confirmed by the characteristic Congo-red staining of laryngeal biopsies. The search for other localizations of amyloidosis was negative. No monoclonal plasma cell proliferation was detected. Both patients received endoscopic CO2 laser excision. In one case, chemotherapy was initially associated due to dystrophic plasma cells in bone marrow aspiration and then withdrawn because of clinical failure. With a 6-month follow-up, hoarseness remained stable.
Laryngeal amyloidosis is an essentially localized disease revealed by hoarseness. Treatment is endoscopic by laser excision. In nearly half of the cases in the literature, it had to be repeated due to localized recurrent lesions. Long-term prognosis of localized laryngeal amyloidosis is better than systemic AL amyloidosis.
喉是淀粉样变性罕见的沉积部位。诊断可能会延迟,在声音长期嘶哑的患者中会被引发。我们报告了两例喉淀粉样变性病例。
一名男性和一名女性分别在1年和3年期间出现声音嘶哑。喉镜检查显示喉部弥漫性浸润。通过喉活检的特征性刚果红染色确诊为淀粉样变性。对淀粉样变性其他部位的检查结果为阴性。未检测到单克隆浆细胞增殖。两名患者均接受了内镜下二氧化碳激光切除术。在一例中,由于骨髓穿刺显示有营养不良性浆细胞,最初联合了化疗,但由于临床效果不佳随后停用。经过6个月的随访,声音嘶哑情况保持稳定。
喉淀粉样变性本质上是一种以声音嘶哑为表现的局限性疾病。治疗方法是内镜下激光切除。在文献报道的近一半病例中,由于局部复发病变,不得不重复治疗。局限性喉淀粉样变性的长期预后优于系统性AL淀粉样变性。