Portal hypertension related to light chain deposition disease of liver: an enlightening experience.

A 55-year-old alcoholic man presented with firm hepatomegaly, ascites and markedly elevated alkaline phosphatase. He had a history of pulmonary tuberculosis. Work-up for malignancy was negative. Histological examination of liver showed extracellular deposition of pink amorphous material which is Congo red stain negative. Deteriorating renal function and nephrotic-range proteinuria were noted. Renal histology showed thickening of the glomerular and tubular basement membranes by non-congophilic deposits along with mesangial expansion. Bone marrow examination revealed patchy areas of pink amorphous deposits which are Congo red stain negative. Immunohistochemical staining of amorphous depositions in liver, kidney and bone marrow were positive for κ light chains. Serum-free light chain assay confirmed markedly elevated free κ-light chain. κ-light chain deposition disease is a systemic disease with universal renal involvement but rarely it presents as chronic cholestatic liver disease with portal hypertension and frequently associated with fatal outcome due to diagnostic delay.