PDF herunterladen
CC BY 4.0 · Indian J Med Paediatr Oncol
DOI: 10.1055/s-0044-1782234
Brief Communication

Upfront Low-Dose Cytarabine with Prednisolone for Langerhans Cell Histiocytosis with Liver Dysfunction: A Ray of Hope

Niranjan Gurunath Hegde 
1   Department of Pediatric Hemato-Oncology and BMT, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India
,
Meena S. Sivasankaran
1   Department of Pediatric Hemato-Oncology and BMT, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India
,
Vimal Kumar G.
2   Department of Pediatric Hemato-Oncology, Immunology and Stem cell transplantation, Dr Rela Institute and Medical Centre, Chennai, Tamil Nadu, India
,
Deenadayalan Munirathnam
2   Department of Pediatric Hemato-Oncology, Immunology and Stem cell transplantation, Dr Rela Institute and Medical Centre, Chennai, Tamil Nadu, India
› Institutsangaben Funding None.
› Weitere Informationen
Auch verfügbar auf
   Lizenzen und Reprints

Abstract

The management of Langerhans cell histiocytosis (LCH) with accompanying liver dysfunction poses significant challenges, and this prompted the development of a modified low-dose cytarabine and prednisolone regimen. In this prospective observational study on children with LCH and liver dysfunction, four patients underwent induction and maintenance chemotherapy. The induction phase included 5 days of 100 mg/m2 cytarabine and 4 weeks of 40 mg/m2 daily prednisolone, with subsequent tapering. Maintenance included a regimen of 5 days of 100 mg/m2 cytarabine, along with oral prednisolone, repeated every 3 weeks. Complete disease resolution occurred after varying chemotherapy cycles. Three patients had liver transplants, and the chemotherapy resumed for 52 weeks after the transplant. In one child, chemotherapy was continued after reaching remission. In conclusion, a modified, less toxic low-dose cytarabine-based chemotherapy effectively managed LCH with liver dysfunction, with liver transplantation as a postremission treatment option.

Keywords

decompensated liver - PET-CT - sclerosing cholangitis - liver transplantation

Author Contributions

We state that all authors have contributed to the manuscript in significant ways, have reviewed and agreed upon the manuscript content.


N.G.H. contributed to the design of the study, literature studies, clinical studies, data acquisition, data analysis, statistical analysis, manuscript preparation, and manuscript editing. M.S.S. contributed to the concept and design of the study, definition of intellectual content, literature studies, manuscript preparation, manuscript editing, and manuscript reviewing. V.K.G. contributed to the concept of the study, definition of intellectual content, manuscript editing, and manuscript reviewing. D.M. contributed to the concept of the study, definition of intellectual content, manuscript editing, and manuscript reviewing.


Patient Consent

Patient consent was obtained.




Publikationsverlauf

Artikel online veröffentlicht:
23. Mai 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • References

  • 1 Yi X, Han T, Zai H, Long X, Wang X, Li W. Liver involvement of Langerhans' cell histiocytosis in children. Int J Clin Exp Med 2015; 8 (05) 7098-7106
    PubMedGoogle Scholar
  • 2 Jaffe R. Liver involvement in the histiocytic disorders of childhood. Pediatr Dev Pathol 2004; 7 (03) 214-225
    CrossrefPubMedGoogle Scholar
  • 3 Braier J, Ciocca M, Latella A, de Davila MG, Drajer M, Imventarza O. Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell histiocytosis. Med Pediatr Oncol 2002; 38 (03) 178-182
    CrossrefPubMedGoogle Scholar
  • 4 Haupt R, Minkov M, Astigarraga I. et al; Euro Histio Network. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 2013; 60 (02) 175-184
    CrossrefPubMedGoogle Scholar
  • 5 Menon J, Shanmugam N, Valamparampil J. et al. Outcomes of liver transplantation in children with Langerhans cell histiocytosis: experience from a quaternary care center. Pediatr Blood Cancer 2023; 70 (01) e30024
    CrossrefPubMedGoogle Scholar
  • 6 Vinblastine: pediatric drug information. In: Post T. ed. UpToDate. Waltham, MA: UpToDate; 2023. Accessed August 13, 2023 at: www.uptodate.com
    Google Scholar
  • 7 Anton W, Giuseppe G, Atkins MB. Goodman & Gilman's The Pharmacological Basis of Therapeutics. New York, NY: McGraw-Hill Education; 2018
    Google Scholar
  • 8 Kaplan KJ, Goodman ZD, Ishak KG. Liver involvement in Langerhans' cell histiocytosis: a study of nine cases. Mod Pathol 1999; 12 (04) 370-378
    Google Scholar
  • 9 Sarin SK, Choudhury A, Sharma MK. et al; APASL ACLF Research Consortium (AARC) for APASL ACLF working Party.. Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific association for the study of the liver (APASL): an update. Hepatol Int 2019; 13 (04) 353-390
    CrossrefPubMedGoogle Scholar
  • 10 Menon J, Rammohan A, Vij M, Shanmugam N, Rela M. Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: a narrative review. World J Gastroenterol 2022; 28 (30) 4044-4052
    CrossrefPubMedGoogle Scholar
  • 11 Donadieu J, Piguet C, Bernard F. et al. A new clinical score for disease activity in Langerhans cell histiocytosis. Pediatr Blood Cancer 2004; 43 (07) 770-776
    CrossrefPubMedGoogle Scholar
  • 12 Minkov M, Rodriguez-Galindo C. Treatment of Langerhans cell histiocytosis: it is time to learn from the past. Br J Haematol 2015; 171 (01) 148-149
    CrossrefPubMedGoogle Scholar
  • 13 Simko SJ, McClain KL, Allen CE. Up-front therapy for LCH: is it time to test an alternative to vinblastine/prednisone?. Br J Haematol 2015; 169 (02) 299-301
    CrossrefPubMedGoogle Scholar
  • 14 Clinicaltrials.gov. Vinblastine/Prednisone versus Single Therapy with Cytarabine for Langerhans Cell Histiocytosis (LCH). Clinicaltrials.gov. Accessed September 13, 2023 at: https://classic.clinicaltrials.gov/ct2/show/NCT02670707
  • 15 Cournoyer E, Ferrell J, Sharp S. et al. Dabrafenib and trametinib in Langerhans cell histiocytosis and other histiocytic disorders. Haematologica 2024; Apr 4; 109 (04) 1137
    PubMedGoogle Scholar
  • 16 Khunger A, Khunger M, Velcheti V. Dabrafenib in combination with trametinib in the treatment of patients with BRAF V600-positive advanced or metastatic non-small cell lung cancer: clinical evidence and experience. Ther Adv Respir Dis 2018; 12: 1753466618767611
    CrossrefPubMedGoogle Scholar