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 hyperbiliru...

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Bibliographic Details
Published in:Therapeutic apheresis and dialysis 2006-02, Vol.10 (1), p.87-89
Main Authors: Song, Kyung Soon, Sung, Hyun Joo
Format: Article
Language:English
Subjects:
Adult
Bile Ducts, Extrahepatic - pathology
Bilirubin - blood
Cholestasis, Intrahepatic - complications
Cytokine
Epstein-Barr Virus Infections - complications
Epstein‐Barr virus
Fatal Outcome
Hemophagocytic syndrome
Humans
Interleukin-6 - blood
Lymphohistiocytosis, Hemophagocytic - blood
Lymphohistiocytosis, Hemophagocytic - therapy
Male
Plasma Exchange
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Summary::  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.
ISSN:1744-9979
1744-9987
DOI:10.1111/j.1744-9987.2006.00347.x