loading page

Outcomes of Liver transplantation in children with Langerhans Cell Histiocytosis: Experience from a quaternary care centre and an algorithmic approach
  • +6
  • Jagadeesh Menon,
  • Naresh Shanmugum,
  • Joseph Valamparampil,
  • Mukul Vij,
  • Vimal Kumar,
  • DEENADAYALAN MUNIRATHNAM,
  • Abdul Hakeem,
  • Ashwin Rammohan,
  • Mohamed Rela
Jagadeesh Menon
Dr Rela Institute and Medical Centre

Corresponding Author:[email protected]

Author Profile
Naresh Shanmugum
Dr Rela Institute and Medical Centre
Author Profile
Joseph Valamparampil
Dr Rela Institute and Medical Centre
Author Profile
Mukul Vij
Dr Rela institute and medical center
Author Profile
Vimal Kumar
Dr Rela Institute and Medical Centre
Author Profile
DEENADAYALAN MUNIRATHNAM
Dr Rela Institute and Medical Centre
Author Profile
Abdul Hakeem
Dr Rela Institute and Medical Centre
Author Profile
Ashwin Rammohan
Dr Rela Institute and Medical Centre
Author Profile
Mohamed Rela
Dr Rela Institute and Medical Centre
Author Profile

Abstract

Objective : Spectrum of hepatic presentation in Langerhans cell histiocytosis (LCH) varies from asymptomatic hepatomegaly to secondary sclerosing cholangitis leading to cirrhosis with or without decompensation. Conventional chemotherapy may be counterproductive in a patient with LCH and hepatic decompensation. We analysed the outcomes of our patients with hepatic presentation of LCH, including their post liver transplant (LT) follow up. Methods: A retrospective analysis was performed on patients with hepatic presentation of LCH referred to our unit. Their clinical profile, chemotherapy protocol, details of LT and survival were analysed. A management algorithm based on the outcomes was proposed. Results: Five of 8 patients were male. Median age of diagnosis was 25(9-48) months. 8(100%) patients had portal hypertension with 4(50%) having decompensated cirrhosis. 6 (75%) patients underwent LT of which 2 had acute decompensation and 4 had sclerosing cholangitis with portal hypertension. Of the two remaining patients, 1 did not tolerate chemotherapy and succumbed, whereas 1 patient after first cycle of chemotherapy was lost to follow up. As their liver disease was worsening during chemotherapy (after 8 & 20 weeks of chemotherapy), two patients underwent urgent LT followed by continuation of chemotherapy. After median follow-up of 30.5 (10.5-50) months, all patients were alive with stable graft function and no disease recurrence. Conclusion: As shown in our series, an algorithmic approach to patient and treatment selection for LCH patients with liver involvement combined with newer chemotherapeutic agents and an optimized immunosuppression can result in excellent outcomes for a hitherto unfamiliar disease.
29 Apr 2022Submission Checks Completed
29 Apr 2022Assigned to Editor
29 Apr 2022Submitted to Pediatric Blood & Cancer
03 May 2022Reviewer(s) Assigned
16 May 2022Review(s) Completed, Editorial Evaluation Pending
16 May 2022Editorial Decision: Revise Major
21 Jul 20221st Revision Received
21 Jul 2022Submission Checks Completed
21 Jul 2022Assigned to Editor
25 Jul 2022Reviewer(s) Assigned
05 Aug 2022Review(s) Completed, Editorial Evaluation Pending
06 Aug 2022Editorial Decision: Revise Minor
06 Sep 20222nd Revision Received
06 Sep 2022Submission Checks Completed
06 Sep 2022Assigned to Editor
06 Sep 2022Review(s) Completed, Editorial Evaluation Pending
06 Sep 2022Editorial Decision: Accept