Cholestasis and liver failure with lambda-AL amyloidosis.

Abstract

Clinically significant liver involvement in systemic amyloidosis, especially with cholestasis, is rare. We report a case of primary amyloidosis with severe intrahepatic cholestasis leading to terminal liver failure. The present case is the first of its kind reported involving Lambda light chains only as the associated paraprotein. Conventional treatment and a therapeutic trial with dimethyl sulphoxide were unsuccessful. The liver is known to be a common site of amyloid deposition, both in primary and secondary amyloidosis.' Hepatocellular failure and portal hypertension are rare, however, with cholestasis and jaundice found in only about 5% of cases.2 In primary amyloidosis with massive liver involvement the paraprotein consists characteristically of Kappa light chains.3 We report a case of primary amyloidosis of Lambda light chains with dominant liver involvement leading to cholestasis and terminal hepatic failure. CASE REPORT A 69 year old dentist was sent for evaluation of hepatomegaly found on a routine medical examination. He was asymptomatic and had been well throughout his life. On examination, a firm, nontender liver was palpated 8 cm beneath the right costal margin. The span was 19 cm on percussion. The tip of the spleen was palpable. Enlarged left axillary and bilateral inguinal lymph nodes were also palpated. The physical examination was otherwise unremarkable. A complete blood count (CBC), prothrombin time (PT), serum electrolytes, calcium, blood urea nitrogen (BUN), creatinine, albumin, globulin, alkaline phosphatase (ALP) and bilirubin were within normal limits.

Amyloidosis

“…In AL amyloidosis involving the liver, the thrombin time is virtually always elevated [59], The coagulation abnor malities are, however, not predictive of bleeding. The risk of bleeding and of spontaneous rupture of the liver is only about 3% [59], In AL amyloidosis, the deposits preferentially exhibit a 'sinusoidal' pattern, in AA they are limited to vessel walls in the portal areas ('vascular pattern') [96,106,107].…”

“…96, 114, 151], jaun dice is rare in hepatic amyloidosis. It occurs predomi nantly in AL type; the heavy chain is mostly IgG, the light chain is characteristically k [96,136,151].…”

“…Both major types of systemic amyloidosis (AL and AA) involve the liver in most cases [59,96,114,145], Liver involvement is more prominent in AA than in AL amyloidosis [72], The liver is also the most frequent extrarenal localization of LCDD [8]. In the senile form, the liver is uncommonly, and in endocrine forms never affected [114].…”

The legend of the lardaceous liver

“…In the 140 years or so since Virchow ordained its appellation, we have learned much about the classification, epidemiology, and natural history of systemic amyloidosis, 19 -21 its pathogenesis and clinical presentations, and diagnosis and treatment 19 -21 that nowadays can be based on what is known about mechanisms at the molecular level. 22,23 The diverse presentations of hepatic amyloidosis have been appreciated 24 to include not only cholestasis, [25][26][27] that seemingly was not seen by the physicians of old, 9,28 but also the complications of portal hypertension 29 and liver failure, 30 alone or in combination. 26,27,30 Even the great William Osler remarked, "There are no characteristic symptoms of this condition.…”

“…22,23 The diverse presentations of hepatic amyloidosis have been appreciated 24 to include not only cholestasis, [25][26][27] that seemingly was not seen by the physicians of old, 9,28 but also the complications of portal hypertension 29 and liver failure, 30 alone or in combination. 26,27,30 Even the great William Osler remarked, "There are no characteristic symptoms of this condition. Jaundice does not occur; the stools may be light-colored, but the secretion of bile persists."…”