Effect of plasma exchange on the circulating IL-6 levels in a patient with fatal hemophagocytic syndrome associated with bile ductopenia.
We report here the case of a patient suffering from hemophagocytic syndrome (HPS) associated with bile ductopenia. A 24-year old man was admitted after suffering fever, sore throat and general malaise for 7 days and jaundice for 2 days. Clinical studies showed hepatic dysfunction with hyperbilirubinemia. Epstein-Barr viral DNA from two bone marrow samples was detected. Bone marrow aspiration disclosed findings of HPS. Liver biopsy showed centrilobular cholestasis with lack of interlobular bile duct. Repeated therapeutic plasma exchange was effective for decreasing serum bilirubin and interleukin-6 levels. The patient received liver transplantation, however, he finally died of alveolar hemorrhage resulting from disseminated intravascular coagulation and acute rejection.
Song
Kyung Soon
KS
Department of Laboratory Medicine, Yonsei University College of Medicine, Seoul, Korea. kssong@yumc.yonsei.ac.kr
Sung
Hyun Joo
HJ
D000328:Adult; D017734:Bile Ducts, Extrahepatic / Q000473:pathology*; D001663:Bilirubin / Q000097:blood; D002780:Cholestasis, Intrahepatic / Q000150:complications; D020031:Epstein-Barr Virus Infections / Q000150:complications; D017809:Fatal Outcome; D006801:Humans; D015850:Interleukin-6 / Q000097:blood*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000628:therapy*; D008297:Male; D010951:Plasma Exchange
D015850:Interleukin-6; D001663:Bilirubin