Multidisciplinary approach to the treatment of cardiac AA amyloidosis and aortic stenosis due to Castleman's disease: A hybrid therapy with tocilizumab and aortic valve replacement

Abstract

A 67-year-old female patient with multicentric Castleman's disease (MCD) was admitted to our hospital with syncope on exertion. She previously had episodes of chronic fatigue, weight loss, prolonged fever, and skin rash accompanied by anemia, polyclonal hypergammaglobulinemia, and elevated inflammatory markers. Computed tomography showed multiple lymphadenopathies. The diagnosis of MCD with cutaneous involvement was made in 2010 based on the clinical manifestations and skin biopsy histopathological findings, which showed massive infiltration of polyclonal plasma cells in the dermis [ 1 Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005; 129: 3-17 Crossref PubMed Scopus (290) Google Scholar , 2 Munoz J. Naing A. Qi M. Kurzrock R. Cutaneous Castleman disease. Br J Haematol. 2012; 157: 652 Crossref PubMed Scopus (5) Google Scholar ]. Comorbidities such as viral infections, collagen diseases, and other malignancies were excluded. She simultaneously had renal impairment because of secondary AA amyloidosis due to MCD, which was diagnosed by renal biopsy based on multiple AA-type amyloid glomerular deposits. Fortunately, following the administration of tocilizumab, a humanized monoclonal interleukin-6 receptor antibody [ [3] Dubrey S.W. Hawkins P.N. Falk R.H. Amyloid diseases of the heart: assessment, diagnosis, and referral. Heart. 2011; 97: 75-84 Crossref PubMed Scopus (136) Google Scholar ], the patient achieved a stable disease status concomitant with improved clinical and laboratory manifestations (decrease in C-reactive protein from 10.0 mg/dL to 0.2 mg/dL and serum creatinine from 3.4 mg/dL to 2.0 mg/dL).