Relapse of liver amyloidosis 6 years after autologous stem cell transplantation

Abstract

44-year-old female presented 7 years ago with ascites and hepatomegaly hence a liver biopsy was performed (Left panel) which showed amyloid deposition replacing most of the hepatic parenchyma. Congo red stain was weakly positive and immunostain for amyloid-associated (AA) protein was negative. Serum protein electrophoresis (SPE) showed a large IgG lambda monoclonal protein (1.8 g/dL) and urine protein electrophoresis (UPE) in 24 hr showed IgG lambda monoclonal protein (0.7 g/V) and concomitant glomerular proteinuria (10.68 g/V). A bone marrow biopsy (middle panel) showed extensive amyloid deposition with 7% monoclonal plasmacytosis also seen with congo red stain (Right panel). Bone survey did not show lytic lesions and an echocardiogram was normal. The patient was diagnosed with AL amyloidosis and underwent high-dose melphalan with autologous peripheral blood stem cell transplantation (PSCT) with partial response in bone marrow biopsy. Six years afterward, liver magnetic resonance imaging (MRI) revealed hepatic recurrence, confirmed by liver biopsy, hence lenalidomide was started which has been well tolerated since then with stable MRI studies. Her current creatinine is normal with her latest UPE showing no monoclonal protein, only mild glomerular proteinuria (1.5 g/V) is seen, while her SPE shows a trace IgG lambda monoclonal protein (<0.1 g/dL). Amyloidosis is a protean entity characterized by extracellular deposition of the abnormal fibril protein named amyloid. The term primary (AL) amyloidosis represents amyloid-light chain deposition in plasma cell neoplasms; whereas secondary (AA) amyloidosis represents amyloid-associated protein deposition in the setting of chronic inflammatory, infectious, or other malignant conditions. Hepatic infiltration by systemic amyloidosis, manifesting with hepatomegaly, is a relatively common finding in this heterogeneous condition [1]. The treatment for AL amyloidosis is chemotherapy and PSCT [3]; whereas, the treatment for AA amyloidosis is aimed toward the associated condition causing abnormal fibril deposition. High-dose chemotherapy and autologous PSCT have demonstrated a sustained hematologic response in systemic AL amyloidosis [2]. Our case documents the fact that hepatic involvement improves in most patients who have complete or partial hematologic response to systemic treatment. Recurrence in the liver may eventually ensue, although it seems that novel agents available for plasma cell neoplasms, including lenalidomide, might have some activity in this setting [4].1 Left panel. Liver biopsy showed amyloid deposition replacing most of the hepatic parenchyma. Middle panel. Bone marrow biopsy showed extensive amyloid deposition with 7% monoclonal plasmacytosis. Right panel. Bone marrow biopsy with congo red stain.