Liver transplantation as rescue treatment in a patient with primary AL kappa amyloidosis

Abstract

Although involvement of the liver is common in systemic amyloidosis, clinical manifestations of hepatic dysfunction and liver biochemical abnormalities are often absent or only mild. Here we report on a patient with primary amyloidosis and rapid development of liver failure, who was successfully treated by liver transplantation. The patient is a 61-year-old Swedish man who was admitted to the local hospital for spontaneous rupture of the spleen. Before admission, he had suffered from diffuse upper abdominal discomfort, diminished appetite, and had lost 15 kg in 6 months.Shortly after splenectomy, he developed cholestatic liver failure with moderate hepatomegaly, jaundice, ascites and hyponatremia. Over a period of 3 weeks his liver failure progressed, renal function deteriorated rapidly, and he developed encephalopathy. Liver transplantation was performed on the 35th day after splenic rupture. Histological examination revealed extensive deposits of arnyloid in the spleen and liver. N-terminal amino acid sequence analysis of the amyloid protein, purified from the patient's native liver, revealed an AL protein of kappa I-type origin. The postoperative course was uncomplicated, apart from one episode of sepsis and one course of treatment for acute rejection. He was discharged from hospital with normal liver function and good kidney function. One year after surgery, he was in good condition, with normal liver function. However, a liver biopsy taken at the same time showed de novo amyloid deposits in the grafted liver. We conclude that liver transplantation may be indicated as a life-saving procedure in rapidly progressing hepatic amyloidosis with cholestatic jaundice, although the underlying disease has not changed.

Treatment of hepatic amyloid light‐chain amyloidosis with bortezomib and dexamethasone in a liver transplant patient

“…Based on our findings, LT may represent a potential salvage treatment option for hepatic AL amyloidosis in the absence of significant secondary extrahepatic disease, and several groups have reported patients with AL amyloidosis who were treated with LT (Table ). However, as LT alone does not affect the production of amyloid protein, additional therapeutic strategies, including chemotherapy and stem cell transplantation, are required after the LT . The indications for LT followed by chemotherapy include patients with liver failure due to AL amyloidosis without any other severe organ dysfunction and patients without an opportunity for stem cell transplantation.…”

Decompensated Liver Disease due to Primary Hepatic Amyloidosis: Is Liver Transplantation Still Mandatory?

“…In 1998 the first case was reported of PHA complicated with liver failure and treated with LT . However, the lack of systemic treatment for AL amyloidosis led to recurrence of the hematologic disease in this reported case and another by Nowak et al With the advent of SCT, three cases of PHA have been managed successfully with LT and subsequent SCT, resulting in control of the underlying hematologic disorder . In our case, although liver disease remained decompensated during CyBorD, achievement of a partial response enabled administration of a conditioning regimen and subsequent SCT, which resulted in complete response.…”

Two cases with hepatic amyloidosis suspected of having primary sclerosing cholangitis

“…Unfortunately, we were unable to suppose the presence of amyloidosis prior to LDLT. Even in liver failure following hepatic amyloidosis, liver transplantation may be indicated as a life‐saving procedure, but the procedure is risky and not necessarily indicated . In this case, treatment policy might have differed if amyloidosis had been diagnosed at an earlier stage and the patient's general condition evaluated adequately.…”