A case of severe dysdialysis due to heart failure complicated with immunoglobulin lambda‒related systemic amyloidosis

Heryon Lee, Fukuoka Kazuhito, Kunitomo Rie, Shimoda Sachiko, Hyodou Chinatsu, Kukimoto Hikaru, Kobayashi Satoshi, Sato Yuriko, Takemori Ai, Miyamoto Ayako, Ozawa Yuko, Isomura Aya, Kawakami Takahisa, Komagata Yoshinori, Kaname Shinya

doi:10.4009/jsdt.55.193

<p>A 75‒year‒old male with a 20‒year history of type I diabetes mellitus was treated for diabetic nephropathy with anti‒glutamic acid decarboxylase antibody positivity, hypertension, diabetic retinopathy, massive proteinuria, and peripheral neuropathy. Later, his renal dysfunction deteriorated； therefore, hemodialysis was started. While he was admitted to hospital for hemodialysis, a rash on his trunk and serum IgA‒λ positivity were observed, and the possibility of systemic amyloidosis was considered, but a gastric endoscopic biopsy showed no amyloid deposition, and echocardiography showed normal function. He started to receive outpatient dialysis at his local clinic, but his blood pressure frequently dropped during the dialysis sessions, and he was often admitted to our hospital due to heart failure. During his third hospitalization, low cardiac function without coronary artery lesions was observed, and the patient suddenly died of ventricular fibrillation. A pathological autopsy was performed to elucidate the cause of death, and findings suggestive of amyloidosis were observed, but immunostaining failed to identify any amyloid precursors. Mass spectrometry led to a diagnosis of amyloid light‒chain amyloidosis with IgA‒λ as the precursor. We experienced a case of systemic amyloidosis with rapid progression of cardiac dysfunction after the initiation of dialysis.</p>

A case of severe dysdialysis due to heart failure complicated with immunoglobulin lambda‒related systemic amyloidosis

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