Fekih L, Boussoffara L, Fenniche S, Hassene H, Romdhane K B, Abdelghaffar H B, Belhabib D, Ayadi A, Zidi A, Megdiche M L
Service de Pneumologie Ibn Nafiss, Hôpital Abderrahmen Mami, Ariana, Tunisie.
Rev Mal Respir. 2011 May;28(5):691-5. doi: 10.1016/j.rmr.2010.09.036. Epub 2011 Apr 9.
Amyloidosis is characterized by tissue deposits of amyloid material. Secondary amyloidosis can occur as a sequel to pulmonary tuberculosis over a relatively long period. However, this was not the case with our patient. Subsequently we conducted a literature review to try to explain the unusual course of AL amyloidosis in our patient.
A 36- year-old patient was admitted to our department for investigation of haemoptysis. A diagnosis of primary pulmonary tuberculosis was made and antituberculous treatment was started. On the second day of treatment, a haematoma appeared on the sole of the right foot, which spread down to the toes during the following days. Renal investigations showed a 24h proteinuria of 9 g/L and serum protein electrophoresis revealed an albumin level of 11.8 g/L. A diagnosis of nephrotic syndrome was made. A renal biopsy was indicated but this was not possible on account of a marked worsening of the patient's condition after 14 days of treatment. The patient's level of consciousness deteriorated and he was transferred to the intensive care unit for ventilation. He died 48 hours later. Post-mortem histological examination of pulmonary and cutaneous tissue revealed AL amyloid deposits.
In view of the association of active pulmonary tuberculosis and a pulmonary localisation of amyloidosis, a causal relationship is not definite. Coexistence of active pulmonary tuberculosis and primary amyloidosis must also be considered, particularly as the immunohistochemical characterisation revealed AL amyloidosis.
淀粉样变性的特征是淀粉样物质在组织中沉积。继发性淀粉样变性可在较长时期内作为肺结核的后遗症出现。然而,我们的患者并非如此。随后,我们进行了文献综述,试图解释我们患者中AL淀粉样变性的不寻常病程。
一名36岁患者因咯血入院接受检查。诊断为原发性肺结核并开始抗结核治疗。治疗第二天,右脚脚底出现血肿,在接下来的几天里蔓延至脚趾。肾脏检查显示24小时蛋白尿为9g/L,血清蛋白电泳显示白蛋白水平为11.8g/L。诊断为肾病综合征。建议进行肾活检,但由于治疗14天后患者病情明显恶化,无法进行。患者意识水平恶化,被转至重症监护病房进行通气。48小时后死亡。肺和皮肤组织的尸检组织学检查显示有AL淀粉样沉积物。
鉴于活动性肺结核与淀粉样变性的肺部定位有关,因果关系并不明确。还必须考虑活动性肺结核与原发性淀粉样变性的共存,特别是因为免疫组织化学特征显示为AL淀粉样变性。