Loss of intrahepatic bile ducts : an important feature of familial hemophagocytic lymphohistiocytosis

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, fatal disorder of early infancy. We report two siblings with FHL whose symptoms were dominated by hepatic failure. Both presented with sudden-onset fever and hepatosplenomegaly with progressive abnormalities of clinical biochemistry indice...

Full description

Saved in:
Bibliographic Details
Published in:Virchows Archiv : an international journal of pathology 2005-06, Vol.446 (6), p.619-625
Main Authors: KAPELARI, Klaus, FRUEHWIRTH, Martin, HEITGER, Andreas, KÖNIGSRAINER, Alfred, MARGREITER, Raimund, SIMMA, Burkhard, OFFNER, Felix Albert
Format: Article
Language:English
Subjects:
Bile Ducts, Intrahepatic - pathology
Biological and medical sciences
Female
Gastroenterology. Liver. Pancreas. Abdomen
Histiocytosis, Non-Langerhans-Cell - complications
Histiocytosis, Non-Langerhans-Cell - pathology
Histiocytosis, Non-Langerhans-Cell - physiopathology
Humans
Immunohistochemistry
Infant
Investigative techniques, diagnostic techniques (general aspects)
Liver
Liver cirrhosis
Liver Failure, Acute - etiology
Liver Failure, Acute - pathology
Liver Failure, Acute - surgery
Liver Transplantation
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Lymphocytes
Male
Medical sciences
Other diseases. Semiology
Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques
Stem Cell Transplantation
Stem cells
Transplants & implants
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, fatal disorder of early infancy. We report two siblings with FHL whose symptoms were dominated by hepatic failure. Both presented with sudden-onset fever and hepatosplenomegaly with progressive abnormalities of clinical biochemistry indices of liver function. One died of hepatorenal failure. The other underwent liver transplantation. Autopsy and explant liver displayed portal and periportal infiltrates of T lymphocytes and histiocytes; an activation of the hepatic mononuclear phagocytic system with focal hemophagocytosis; and almost complete loss of interlobular bile ducts. Paucity of bile ducts dominated in a pre-transplant liver biopsy specimen (and transiently obscured the diagnosis of FHL). Disease recurred in the allograft, again with lymphohistiocytic infiltration and destruction of interlobular bile ducts. Consequently the patient underwent haploidentical peripheral stem cell transplantation. This patient is alive 5 years later. Loss of bile ducts may be an important feature of hepatic involvement by FHL.
ISSN:0945-6317
1432-2307
DOI:10.1007/s00428-005-1238-y