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Diagnosis of a seriously ill patient misses the link between a type of hepatitis and Hodgkin's disease

This clinical case of a 46-year-old Mexican immigrant, who arrived at the hospital with epigastric pain and vomiting of coffee-grounds material, points out the importance of considering a diagnosis of Hodgkin's disease in any feverish patient with unexplained intrahepatic cholestasis (vanishing bile duct syndrome). The patient suffered from fatigue, malaise, jaundice, and a 20-lb weight loss during the past 2 months. Due to the insidious nature of this patient's illness, fever, enlarged lymph nodes, and history of exposure to livestock, the physicians treated him for brucellosis (a livestock infection that can be transmitted to humans).

A week later, he was readmitted to the hospital, but had no dilation of the pancreatic or liver bile ducts. The patient's neutropenia (decrease in a type of infection-fighting white blood cell), which may have been a manifestation of worsening liver disease, is also associated with brucellosis and tuberculosis (which he was treated for next). The patient was readmitted 2 weeks later with elevated white cell count and enlarged lymph nodes in the chest and abdomen. The patient was discharged while taking prednisone for presumed granulomatous hepatitis. A month later, the patient returned to the clinic with persistent weight loss and abdominal pain. Liver tests showed a badly damaged liver and the liver biopsy specimen revealed a rapidly progressive intrahepatic cholestatic process (failure of bile to flow from the liver ducts). The doctor remained stumped at this point.

After a progressive downhill course, the patient died. At autopsy, a diagnosis of Hodgkin's disease was made. A review of all previous biopsy specimens showed no evidence of Hodgkin's disease. Cholestatic hepatitis has been described in several case reports as appearing several weeks before the Hodgkin's disease became clinically apparent. Although Hodgkin's disease reportedly has remained undiagnosed after an initial bone marrow biopsy, the physicians were not aware of reports of a similar difficulty in detecting it after numerous lymph node and bone marrow biopsies, as in this case. The study was supported in part by the Agency for Healthcare Research and Quality (HS11540).

More details are in "Empirically incorrect," by Amy Schmitt, M.D., Daniel J. Gilden, M.D., Sanjay Saint, M.D., M.P.H., and Richard H. Moseley, M.D., in the February 2, 2006, New England Journal of Medicine 354(5), pp. 509-514.

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