Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell Histiocytosis.

Objective: To analyze features and outcomes of cholestasis, sclerosing cholangitis (SC), and liver transplantation (LTx) in patients with Langerhans cell Histiocytosis (LCH) between October 1987 and June 1999.

Study Design: Of 182 cases with LCH, 36 had hepatic involvement and 12 of those presented with cholestasis. These 12 were the focus of our study. Their median age was 23 months (range: 3-36). Hepatomegaly or hepatosplenomegaly was found in 11 of the 12; elevations of alkaline phosphatase, transaminases, gamma glutamyl transpeptidase (GGT), and less frequently direct bilirubin were detected. Sonography, liver biopsy, and cholangiography were consistent with the diagnosis of SC in 11 patients. None of the biopsies revealed Langerhans cells (LC). Frequently associated lesions of skin, bone, and ear were noted. Early patients were treated with Vinblastine/prednisone for 8 weeks, later patients with the LCH I and LCH II protocols of the Histiocyte Society (HS).

Results: Median follow-up was 28 months (range: 10-86). Three patients improved and remained without signs of progressive SC at 27, 32, and 86 months. Nine had progressive liver sequelae resistant to chemotherapy. Of these nine, five received LTx, three died before LTx with progressive SC, and one awaits LTx. Three LTx patients survive without disease reactivation 14, 25, and 37 months post-transplant. Two patients died less than one month after LTx, due to renal failure and sepsis in the first patient and bowel volvulus with perforation followed by sepsis in the second one.

Conclusions: SC is a frequent and usually progressive sequela of multisystem LCH in our institution. LTx has become the treatment of choice for the majority of patients and should be considered early in cases with severe hepatic involvement.