Plasmaphoresis and plasma exchange in the treatment of hyperlipaemia and xanthomatous neuropathy in patients with primary biliary cirrhosis

Abstract

SUMMARY Two patients with primary biliary cirrhosis who were increasingly incapacitated by xanthomatous neuropathy are described. Treatment with a low fat diet and cholestyramine was unhelpful but repeated plasmaphoresis by simple venesection in one and plasma exchange using an IBM blood cell separator in the other over a period of several months completely relieved the symptoms of the neuropathy, caused skin xanthomata to recede, and lowered plasma lipid levels in both patients. There was no evidence that this procedure was associated with any deleterious effects on the liver. The size of the cholesterol pool in xanthomata in one patient was estimated to be approximately 35 g, and from the plasma cholesterol response to plasmaphoresis at varying frequency it was suggested that the excess of cholesterol synthesis over degradation was less than 0.3 g/day in one patient and less than 0.4 g/day in the other. On the basis of the response in these patients it is suggested that the turnover rates of lipid pools are relatively slow in biliary cirrhosis and that cholesterol accumulation is more likely to be due to a reduced catabolic rate than to an increased synthetic rate of cholesterol. Plasmaphoresis or plasma exchange are useful methods of treatment for the rare patient afflicted by this resistant and distressing complication of biliary cirrhosis.

Clinical, biochemical, and immunohistochemical features of necrobiotic xanthogranulomatosis

“…The burning pain experienced in the lesions may be the result of neural involvement, which can be a feature of other florid xanthomata, occurring in-for example, type V hyperlipoproteinaemia 24 and primary biliary cirrhosis. 25 As new cases of NXG arise, immunohistochemistry of fresh biopsy material for metalloproteinases may provide further insight into its pathogenesis and may also contribute to our understanding of atheromatous plaque rupture. (1) indicates the start of the glycine elution.…”

Therapeutic apheresis in special populations

“…Primary biliary cirrhosis (PBC) is a condition that leads to accumulation of cholesterol. Turnberg et al showed successful clearance of cholesterol and relief of xanthomatous neuropathy by plasmapheresis [17]. There is no good evidence to confirm that accumulation of bile salts in PBC is reduced with plasma exchange.…”

Novel strategies and therapeutic options for the management of primary biliary cholangitis

“…Plasmapheresis has been suggested as a treatment for resistant complications of PBC going as far back as the 1970s. 80,81 However, its use in pruritus related to cholestasis is still in the experimental phase. A recent study of 17 patients with PBC with refractory pruritus and who received 129 sessions of plasmapheresis over 40 hospital admissions has been published.…”