Rare case of acute on chronic hepatic failure in a patient with multiple myeloma-associated amyloidosis

Abstract

Amyloidosis is the extracellular deposition of unique protein fibrils in different tissue organs. It is most commonly associated with B-cell malignancy such as multiple myeloma or Waldenstrom macroglobulinaemia. It involves the liver, heart, kidney, peripheral nerves and soft tissues. Liver however is affected, but clinically apparent disease is very rare. Hepatomegaly and mild elevation of alkaline phosphatase is the most common presentation in patients with liver involvement. Acute hepatic failure is a rare presentation with myeloma-induced amyloidosis. The diagnosis can be difficult requiring biopsy or sometimes special staining of the tissue. Management is still very challenging.

Keywords: gastroenterology; liver disease; oncology.

Figure 1

Ultrasound of abdomen demonstrating normal flow in hepatic artery and perihepatic ascites (white arrow).

Figure 2

Liver biopsy demonstrating diffuse sinusoidal amyloidosis (black arrow) - congophilic, extracellular, pale eosinophilic, hyaline, amorphous, acellular material.

Figure 3

Immunostain for CD-138 for palsma cells in bone marrow.

Figure 4

Bone marrow biopsy - Congo red stain light micrograph with salmon pink staining for amyloid.

Figure 5

Bone marrow biopsy- Congo red stain (polarised light micrograph) with apple green birefringence for amyloid.

Figure 6

Algorithm for diagnosis of multisystem amyloidosis.