Finsterer Josef, Höftberger Romana, Stöllberger Claudia, Wöhrer Adelheid, Regele Heinz
Krankenanstalt Rudolfstiftung, Vienna, Austria.
J Oncol Pharm Pract. 2013 Jun;19(2):170-4. doi: 10.1177/1078155212443991. Epub 2012 Apr 13.
Restrictive cardiomyopathy due to AL amyloidosis has not been reported as the cause of sudden death. The risk of sudden death in AL amyloidosis may be further increased by potentially cardiotoxic medication, as in the following case.
In a 69-year-old female, AL amyloidosis from light-chain deposition disease manifested as gastrointestinal pseudo-obstruction, restrictive cardiomyopathy, and secondary myopathy. AL amyloidosis was histologically confirmed by endomyocardial biopsy and muscle biopsy. One month after initiation of steroids and lenalidomide the patient suddenly died during sleep. It is speculated that sudden death was due to restrictive cardiomyopathy, cardiotoxicity of lenalidomide, pulmonary embolism, sudden unexplained death in epilepsy syndrome or stroke. The possible causes of sudden death are discussed.
This case shows that AL amyloidosis from light-chain deposition disease may predominantly affect the intestines, myocardium and the skeletal muscle and that lenalidomide may have a beneficial effect on the amyloidosis but should be given with caution for its potential arrhythmogenic and thrombogenic side-effects.
由AL型淀粉样变性引起的限制性心肌病尚未被报道为猝死的原因。如下例所示,潜在的心脏毒性药物可能会进一步增加AL型淀粉样变性患者猝死的风险。
一名69岁女性,轻链沉积病所致的AL型淀粉样变性表现为胃肠道假性梗阻、限制性心肌病和继发性肌病。经心内膜心肌活检和肌肉活检组织学确诊为AL型淀粉样变性。在开始使用类固醇和来那度胺治疗1个月后,患者在睡眠中突然死亡。推测猝死原因是限制性心肌病、来那度胺的心脏毒性、肺栓塞、癫痫综合征或中风导致的不明原因猝死。对猝死的可能原因进行了讨论。
该病例表明,轻链沉积病所致的AL型淀粉样变性可能主要累及肠道、心肌和骨骼肌,来那度胺可能对淀粉样变性有有益作用,但因其潜在的致心律失常和血栓形成副作用,应谨慎使用。