Diving in the big blue: Atypical Mott cells in rapidly fatal primary amyloidosis

[摘要] A 75-year-old man with no previous medical history presented atthe emergency unit with asthenia, anorexia, weight loss, and progressively increasing dyspnea revealing acute heart failure (N-terminal prob-type natriuretic peptide:16 667 ng/L; troponemia: 47 ng/L). Echocardiography showed a granular appearance of the myocardium andinterventricular septal hypertrophy, suggestive of amyloidosis. Chemistry disclosed high levels of immunoglobulin kappa light chains at1240 mg/L (lambda light chains 14 mg/L; κ/λ ratio 86) and a detectablebut unquantifiable IgA κ peak. Myocardial biopsies pathology showedan endocardium thickened by fibrosis. Within the myocardium, thepresence of interstitial fibrillary deposits stained by Congo red andbirefringent in polarized light confirmed the diagnosis of cardiac amyloid light-chain (AL) amyloidosis. The search for other organ damagedisclosed vegetative involvement with orthostatic hypotension, ictericcholestasis (bilirubin 51.8 mg/L), undocumented digestive involvement(diarrhea with evocative endoscopic lesions), and glomerulonephritis(urinary proteinuria 4 g/24 h with 3 g/24 h albumin and 1 g/24 himmunoglobulin light chains).

[关键词] amyloidosis;mott cell;plasma cell