Relapsing hairy cell leukemia presenting as fulminant hepatitis

We report the first known case of fulminant hepatitis due to hairy cell infiltration.

A 43-year-old woman with hairy cell leukemia returned 1 year after diagnosis with spider angiomata of the face and neck, palmar erythema, and diffuse telangiectasias. The liver span was normal. Laboratory studies included: aspartate aminotransferase, 56 U/L; alkaline phosphatase, 283 U/L; gamma-glutamyltransferase, 157 U/L; and normal bilirubin. A liver biopsy demonstrated modest infiltration of hairy cells with hemosiderosis and intact hepatocytes.

The patient responded to interferon-alpha with resolution of the angiomata, decreased palmar erythema, and improved liver function tests. Five months later, she presented with right upper quadrant pain, anorexia, fatigue, and diffuse myalgias. The physical examination was unchanged. Laboratory studies included: aspartate aminotransferase, 299 U/L; alkaline phosphatase, 1,388 U/L; total bilirubin, 43 mumol/L; and direct bilirubin, 29 mumol/L. Two weeks later, she was admitted to the hospital. Her liver span was 15 cm. Laboratory studies showed the following values: aspartate aminotransferase, 618 U/L; alkaline phosphatase, 2,319 U/L; total bilirubin, 311 mumol/L; direct bilirubin, 233 mumol/L; and alanine aminotransferase, 462 U/L. Intensive investigation of contributing causes was not revealing. A subsequent liver biopsy demonstrated extensive portal and intralobular hairy cell infiltration with loss of normal architecture. Liver function deteriorated as shown by the following values: aspartate aminotransferase, 1,100 U/L; alkaline phosphatase, 3,645 U/L; bilirubin, 477 mumol/L; and ammonia, 47 mumol N/dL. The patient died 9 days after admission.

Although liver infiltration is often present and mild elevations of liver function tests have been noted, evidence of such extensive hepatic injury caused by hairy cell leukemia has not been reported previously. Hairy cell infiltration of the liver can cause typical cutaneous changes of liver disease and even fulminant hepatitis.