Hepatic Amyloidosis (AL): The Natural History in 80 Patients
We followed 80 patients with liver biopsy-proven primary amyloidosis (AL) to study its natural history. Liver biopsy does not carry a major risk of bleeding or rupture. Although the liver dominated the clinical presentation, most patients had evidence of systemic involvement. Overall, 77 percent had an associated nephrotic syndrome, congestive heart failure, or neuropathy. The history and physical examination was not helpful in differentiating hepatic amyloidosis from other forms of liver disease. Laboratory studies that suggested the diagnosis were proteinuria (88 percent) and either a monoclonal band or hypogammaglobulinemia on serum protein electrophoresis (64 percent) or hyposplenism on the peripheral blood smear (51 percent). The value of protein electrophoresis was enhanced by immuno-electrophoresis of the serum and urine. When this was done, a monoclonal protein was detected in 76 percent. Kappa light chains were not associated with giant hepatomegaly. Hepatomegaly occurred in the absence of abnormal liver function tests (32 percent). Although the alkaline phospha-tase at diagnosis had no impact on survival, a two-fold elevation in SGOT or bilirubin predicted a median survival of four months. Myeloma was diagnosed in 12 but had no effect on the clinical course. The commonest coagulation abnormality was prolongation of the thrombin time. No coagulation parameter predicted bleeding risk. The median survival was nine months with projected five-and ten-year survivals of 13 percent and 1 percent. Five patients had indolent disease surviving 60+ months. These survivors had no distinguishing features that permitted their recognition prospec-tively. There was no impact of treatment on the course of the disease.
KeywordsLiver Biopsy Nephrotic Syndrome Carpal Tunnel Syndrome Thrombin Time Peripheral Blood Smear
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