Liver involvement in Langerhans' cell histiocytosis: A study of nine cases

Citation
Kj. Kaplan et al., Liver involvement in Langerhans' cell histiocytosis: A study of nine cases, MOD PATHOL, 12(4), 1999, pp. 370-378
Citations number
29
Categorie Soggetti
Research/Laboratory Medicine & Medical Tecnology","Medical Research Diagnosis & Treatment
Journal title
MODERN PATHOLOGY
ISSN journal
08933952 → ACNP
Volume
12
Issue
4
Year of publication
1999
Pages
370 - 378
Database
ISI
SICI code
0893-3952(199904)12:4<370:LIILCH>2.0.ZU;2-U
Abstract
Nine cases of Langerhans' cell histiocytosis (LCH) of the liver are present ed. Five of the patients had liver involvement only. Other organ systems, n otably the lymph nodes and skin, were involved in the other four patients. Four of the patients had sclerosing biliary disease with infiltration of th e bile ducts by Langerhans' cells, whereas in two other patients, the bilia ry sclerosis was not associated with direct hepatic involvement by Langerha ns' cells. Histologically, the lesions were composed of focal aggregates of Langerhans' cells in a polymorphous background of mature eosinophils, lymp hocytes, neutrophils, and plasma cells. LCH encompasses a syndrome that has a broad range of clinical presentations and that might involve the liver s olely as tumor-like lesions or cystic lesions, or as part of systemic disea se. Even when Langerhans' cells are not demonstrable, sclerosing cholangiti s can be seen in LCH.