[AL amyloidosis associated with multiple myeloma revealed by cholestatic hepatomegaly: A case report].

Abstract

INTRODUCTION AL amyloidosis is a rare disorder characterized by the extracellular deposition of immunoglobulin light chain fibrils produced by a clonal plasma cell population. Hepatic involvement as the initial manifestation is rare, and forms associated with jaundice and cholestasis carry a poor prognosis. CASE REPORT We report the case of a 53-year-old man from Togo presenting with massive hepatomegaly, cholestatic jaundice, and weight loss. After a negative etiological workup, diffuse hepatic infiltration associated with a plasma cell dyscrasia raised suspicion of AL amyloidosis. Peripheral biopsies were negative. The diagnosis was confirmed by transjugular liver biopsy, showing κ light chain amyloid deposits associated with a monoclonal plasma cell proliferation. Despite anti-clonal therapy using the ANDROMEDA protocol, the clinical course was rapidly fatal. CONCLUSION This case highlights the critical importance of early diagnosis when facing unexplained cholestatic hepatomegaly.