AL type renal amyloidosis with cardiac involvement: A case report and literature review

AL型肾淀粉样变性伴心脏受累:病例报告及文献综述

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Abstract

RATIONALE: Systemic amyloidosis refers to a spectrum of diseases characterized by extracellular deposition of amyloids, with commonrenal involvement; however, simultaneous involvement of cardiac amyloidosis remains rare. The purpose of this report was to enhance the understanding of the diagnosis and treatment of light chain (AL)-type renal amyloidosis with cardiovascular involvement, reduce misdiagnosis and missed diagnosis, and enable timely and effective treatment for such patients. PATIENT CONCERNS: A 74-year-old male patient was admitted to our hospital because of recurrent edema for over 1 year and aggravated recurrence with decreased urine for 1 month. Pathological examination of the renal biopsy specimen showed homogeneous nonstructural material deposition in the glomerular mesangial area. DIAGNOSES: This patient was diagnosed with lightchain (AL)-type renal amyloidosis with cardiac involvement. INTERVENTIONS: Treatment with daretomumab (DARA) 800mg plus cyclophosphamide 0.4g, bortezomib 1.6mg and dexamethasone 20mg (D-VCD regimen). Interventions, such as intermittent diuresis, anticoagulation, and electrolyte imbalance, were administered. OUTCOMES: After 2 courses of chemotherapy, edema was relieved, urine protein was reduced, and symptoms improved. Chemotherapy was continued in accordance with the regimen described above. LESSONS: Due to the rarity and nonspecific symptoms, missed diagnosis and misdiagnosis of AL-type renal amyloidosis remain common. Among patients with a confirmed diagnosis, in addition to considering the pathological changes in the kidney, consideration of the presence of amyloidosis in other organs, such as cardiac involvement, is also necessary.

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