Results :
Of the 8 patients with a diagnosis of LCH, active disease was present in
6, and 2 patients were referred after completion of chemotherapy with
cirrhosis and portal hypertension. (Table 1). The median age of onset of
symptoms of LCH was 13.5 months (3 to 26 months). The median age of
diagnosis was 25 months and the median interval between the onset of
symptoms of LCH and its diagnosis was 10 months (1 to 15 months).
The common clinical presentations of LCH included hepatomegaly (100%),
scaly lesions of the scalp (75%), skin rash (75%), splenomegaly (75%)
and lytic bone lesions (50%). Liver biopsy revealed features of large
duct obstructive cholangiopathy with portal fibrous expansion, and bile
ductular proliferation without active infiltration by Langerhans cells
One patient had Langerhans cells which were positive for CD1a . The
median PELD score of patients with active disease was 21 (4 – 31.4) and
those with burnt-out disease was 2.2.
The flow of patients is depicted in Figure1. 4 patients each were
fitting into Category 1(Patients 1,2,3 and 4) and Category 2 (Patients
5,6,7 and 8) (Table 1) as per the previously mentioned classification.
In category 1, patient 1 and 2 who had completed chemotherapy 95 and 183
months prior, presented with compensated cirrhosis and portal
hypertension. They underwent FDG-PET screening to make sure there is no
active disease and were offered LT. Patient 3 and 4 with active LCH and
compensated cirrhosis were started on standard Vinblastine based
chemotherapy. They tolerated the chemotherapy and underwent LT after
completion course of drug regimen.
All patients in Category 2, i.e Patients 5,6,7 and 8 had active disease.
Patient 5 was started on standard Vinblastine based protocol and child
had deterioration of his liver function and died of gram negative
sepsis. We changed our policy to use low dose Cytarabine instead of
Vinblastine, which is less hepatotoxic for category 2 patients. The
modified regimen was subsequently started in 3
patients.18 Of the three patients, one (patient 8)
defaulted therapy and was lost to followup. Patient number 6 achieved
remission after 1 cycle of induction and patient number 7 achieved
remission after 3 cycles of induction therapy. Both underwent liver LT
and then completed one year maintenance phase of chemotherapy (Table 1).
Hence 2 out of 6 patients underwent chemotherapy in the post LT period.
The median time of LT from time of diagnosis was 28 months (2 – 195
months) for the whole cohort. Median Duration of ICU stay and hospital
stay were 4 & 12 days respectively. There were no episodes of vascular,
biliary or immunological complications.
The liver explants showed greenish discolouration macroscopically
(Figure 3E, 3F) Light microscopy revealed biliary pattern cirrhosis with
ulceration in large ducts, biliary sludge and duct loss (Figure 3G, 3H).
No significant CD1a or CD 207 positive Langerhans cells were identified
in the explants – indicating LCH in remission
Post-LT patient and graft survival was 100% at a median follow-up of 36
months (18 to 80 months). None of the patients had recurrence of LCH in
the graft or extrahepatic sites.