Liver cirrhosis in histiocytosis X

Abstract

ABSTRACT— Cirrhosis of the liver was diagnosed in an 18‐year‐old man with histiocytosis X. Electron microscopy and immunohistochemistry revealed infiltration of the liver by the typical cellular elements of histiocytosis X. Endoscopic retrograde cholangiography showed alterations resembling those of sclerosing cholangitis. Sixteen months later, the patient died of recurrent variceal bleeding and cholangiosepsis. Autopsy confirmed that cirrhosis was the main manifestation of the underlying disease. Thus, cirrhosis of liver can be a main and potentially fatal manifestation of histiocytosis X beyond the pediatric age range. Histiocytosis X may lead to parenchymal infiltration of the liver and to changes of the major bile ducts resembling sclerosing cholangitis. The diagnosis of hepatic histiocytosis X can easily be missed without relying on appropriate electron microscopic and immunohistochemical investigations.

Case Report Langerhans' Cell Histiocytosis Presenting with Hepatic Dysfunction

“…Conversely , the presence of 8-100-staining cells within portal tracts is not unique to LCH (12). Mural infiltration of large bile ducts by Langerhans ' cells with progressive ductal scarring produces the cholangiographic pattern of sclerosing cholangitis (4,5,10). Xanthomatous inflammation of the large intrahepatic bile ducts would not be detected by liver biopsy alone and could produce the portal fibrosis and ductal proliferation often observed in the liver of LCH patient s with hepatic dysfunction, as seen in this patient.…”

“…Primary sclerosing cholangitis and LCH may have similar radiographic findings. Although ectasia and stenosis limited to the intrahepatic bile ducts are more typical of LCHassociated sclerosing cholangitis, sufficient overlap with primary sclerosing cholangitis exists to preclude a definitive diagnosis based solely upon the cholangiogram (5,(7)(8)(9)(10)(11).…”

“…Hepatomegaly alone does not portend a poor prognosis nor does it correlate well with histologic findings in the liver (4). Liver dysfunction, characterized by cholestasis ,jaundice, pruritus, and/or portal hypertension, usually develops over months or years (3)(4)(5)(6). A hepatic presentation with rapid progression of hepatic dysfunction as manifested by the patient is unusual (7).…”

Adult Langerhans Cell Histiocytosis with Independently Relapsing Lung and Liver Lesions That was Successfully Treated with Etoposide

“…Liver involvement in adult LCH is relatively rare (6)(7)(8). Nagai et al reported 1 case of adult LCH with liver involvement (8) and reviewed 5 previously reported adult cases (9)(10)(11)(12)(13). Interestingly, in their case, radiographic findings of both the lungs and the liver displayed marked improvements after smoking cessation; the case was described as "PLCH with liver involvement".…”

Hepatic Langerhans cell histiocytosis: A review

“…In late stages, patients may present with severe sclerosing cholangitis and liver failure. A subset of patients progresses to develop cirrhosis, which may lead to portal hypertension and secondary hypersplenism[ 22 , 23 ].…”