Portal lymphadenopathy associated with lipofuscin in chronic cholestatic liver disease.

Abstract

To determine whether portal lymphadenopathy in primary biliary cirrhosis is caused by deposition of lipofuscin pigment in sinus histiocytes and to compare primary biliary cirrhosis with other liver diseases a retrospective study on a consecutive series of 169 livers obtained at transplantation was carried out. There were grouped into eight diagnostic categories: primary biliary cirrhosis (n = 51), primary sclerosing cholangitis (n = 10), extrahepatic biliary atresia (n = 6), chronic rejection (n = 9), cirrhosis (other causes) (n = 38), primary liver neoplasia (n = 21), acute liver disease (n = 20), and retransplantation (other) (n = 14). Lymph nodes were present in 66 specimens. Fifty of these contained granules of lipofuscin pigment. The highest incidence of lymph node enlargement and the largest amounts of pigment were present in cases of primary biliary cirrhosis. A similar pattern oflymph node enlargement was also commonly observed in other chronic cholestatic conditions (primary sclerosing cholangitis, biliary atresia, chronic rejection). Much less pigment was seen in nodes draining livers with non-cholestatic cirrhosis or primary tumours. Nodes were not found in acute liver disease. It is concluded that portal lymphadenopathy associated with lipofuscin is a common finding in various chronic cholestatic liver diseases. The pathogenesis of this lesion is uncertain. Most cases are asymptomatic with enlarged nodes which may be detected only at laperotomy or necropsy and may be wrongly attributed to neoplastic disease. Diagnostically, the finding of large amounts of lipofuscin in enlarged portal lymph nodes is a good indicator of underlying chronic cholestatic liver disease. Enlarged, pigmented portal lymph nodes are often seen in end-stage primary biliary cirrhosis. The pigment, initially thought to be bile,' has recently been shown to have the staining characteristics oflipofuscin in two cases reported by Benbow and McMahon.2 To investigate this phenomenon further and to compare primary biliary cirrhosis with other liver diseases we carried out a retrospective histological and histochemical study on lymph nodes attached to livers obtained at liver transplantation.

Hilar lymph nodes sampling at the time of liver transplantation for hepatocellular carcinoma: to do or not to do?.

“…The prognostic value of hilar LN metastases in LT for HCC and the worth of LN sampling are tasks of great importance that have not been previously reviewed systematically. In many cases, transplant surgeons do not perform a hilar LN sampling during the hepatectomy stage of LT. Reasons for such approach include: (i) The LN enlargement in cases of hepatitis C‐induced liver cirrhosis cannot usually be distinguished clinically or radiologically from tumor involvement [9–15, 57], (ii) LT is an ‘emergency‐not scheduled’ operation and in many transplant centers there may be no pathologist on duty during the night or week‐end to perform frozen sections, (iii) ‘Time is running’– a rapid hepatectomy is preferred in order to minimize the cold ischemic time.…”

Developmental abnormalities and liver disease in childhood

“…The median maximum node dimension in six cases studied by Hübscher and Harrison was 14 mm. 302 These lymph nodes are brown in colour and full of pigment-laden macrophages. Livers removed at transplantation after an apparently successful Kasai procedure (loss of jaundice), but with subsequent development of cirrhosis and portal hypertension, are often coarsely nodular with areas of macronodular hypertrophy and broad intervening or peripherally located scars resembling the gross appearance of focal nodular hyperplasia ( Fig.…”

Peculiarities of human pancreatolienal hemolymph node structure and cellular composition.

“…В других органах подобные клетки выявляются при заболеваниях, связанных с нарушением обмена липидов, как врожденных (например, Болезнь Ниманна-Пика) [23], так и приобретенных (длительное парентеральное питание) [ [25]. Также накопление липофусцина в макрофагах регионарных лимфоузлов было выявлено при хронических заболеваниях печени [26], что свидетельствует о возможных альтернативных, независимых от эритрофагоцитоза, причинах накопления пигмента старения в лимфоузлах. Несмотря на эти наблюдения, макрофаги, переполненные липофусцином, в гемолимфатических узлах встречались намного чаще, чем в обычных, что можно объяснить присутствием большого количества ионов железа, высвобождающихся при распаде гемоглобина.…”