Langerhans cell histiocytosis

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Ronald S. Go, MD
Rochester, MN
9 regimens on this page
9 variants on this page


Note: the majority of the regimens on this page are intended for multi-system Langerhans cell histiocytosis (MS-LCH); many patients with other forms of LCH, such as pulmonary (PLCH) do not necessarily need antineoplastic treatment.

Guidelines

Euro-Histio-Net

Untreated

Etoposide & Prednisone

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Regimen

Study Evidence Comparator Efficacy
Gadner et al. 2001 (LCH-I) Phase III (E) Vinblastine & Prednisone Seems not superior

To be completed

Chemotherapy

References

  1. Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, Komp D, Michaelis J, Nicholson S, Pötschger U, Pritchard J, Ladisch S; Histiocyte Society. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr. 2001 May;138(5):728-34. Erratum in: J Pediatr 2001 Jul;139(1):170. link to original article PubMed

Etoposide, Vinblastine, Prednisone

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Regimen

Study Evidence Comparator Efficacy
Gadner et al. 2007 (LCH-II) Phase III (E) Vinblastine & Prednisone Seems not superior

Total duration of therapy is 24 weeks.

Chemotherapy, initial

6-week course, followed by:

Chemotherapy, continuation

21-day cycle for 6 cycles

References

  1. Gadner H, Grois N, Pötschger U, Minkov M, Aricò M, Braier J, Broadbent V, Donadieu J, Henter JI, McCarter R, Ladisch S; Histiocyte Society. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood. 2008 Mar 1;111(5):2556-62. Epub 2007 Dec 18. link to original article contains verified protocol PubMed

Methotrexate, Vinblastine, Prednisone

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Regimen

Study Evidence Comparator Efficacy
Gadner et al. 2013 (LCH-III) Phase III (E) Vinblastine & Prednisone Seems not superior

To be completed

Chemotherapy

References

  1. Gadner H, Minkov M, Grois N, Pötschger U, Thiem E, Aricò M, Astigarraga I, Braier J, Donadieu J, Henter JI, Janka-Schaub G, McClain KL, Weitzman S, Windebank K, Ladisch S; Histiocyte Society. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood. 2013 Jun 20;121(25):5006-14. Epub 2013 Apr 15. link to original article PubMed

Vinblastine & Prednisone

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Regimen

Study Evidence Comparator Efficacy
Gadner et al. 2001 (LCH-I) Phase III (E) Etoposide & Prednisone Seems not superior
Gadner et al. 2007 (LCH-II) Phase III (C) Etoposide, Vinblastine, Prednisone Seems not superior
Gadner et al. 2013 (LCH-III) Phase III (C) Methotrexate, Vinblastine, Prednisone Seems not superior

Total duration of therapy is 24 weeks, in LCH-II.

Chemotherapy, initial

6-week course, followed by:

Chemotherapy, continuation

21-day cycle for 6 cycles

References

  1. Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, Komp D, Michaelis J, Nicholson S, Pötschger U, Pritchard J, Ladisch S; Histiocyte Society. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr. 2001 May;138(5):728-34. Erratum in: J Pediatr 2001 Jul;139(1):170. link to original article PubMed
  2. Gadner H, Grois N, Pötschger U, Minkov M, Aricò M, Braier J, Broadbent V, Donadieu J, Henter JI, McCarter R, Ladisch S; Histiocyte Society. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood. 2008 Mar 1;111(5):2556-62. Epub 2007 Dec 18. link to original article contains verified protocol PubMed
  3. Gadner H, Minkov M, Grois N, Pötschger U, Thiem E, Aricò M, Astigarraga I, Braier J, Donadieu J, Henter JI, Janka-Schaub G, McClain KL, Weitzman S, Windebank K, Ladisch S; Histiocyte Society. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood. 2013 Jun 20;121(25):5006-14. Epub 2013 Apr 15. link to original article PubMed

Relapsed or refractory

Cladribine monotherapy

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Regimen

Study Evidence
Weitzman et al. 2009 (LCH-S-98) Phase II

To be completed

Chemotherapy

References

  1. LCH-S-98: Weitzman S, Braier J, Donadieu J, Egeler RM, Grois N, Ladisch S, Pötschger U, Webb D, Whitlock J, Arceci RJ. 2'-Chlorodeoxyadenosine (2-CdA) as salvage therapy for Langerhans cell histiocytosis (LCH): results of the LCH-S-98 protocol of the Histiocyte Society. Pediatr Blood Cancer. 2009 Dec 15;53(7):1271-6. link to original article PubMed

Cladribine & Cytarabine

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Regimen

Study Evidence
Donadieu et al. 2015 Phase II

Chemotherapy

  • Cladribine (Leustatin) 9 mg/m2 IV over 2 hours once per day on days 2 to 6, should not be administered simultaneously with cytarabine
    • Children weighing less than 10 kg received a dose of 0.3 mg/kg/day
  • Cytarabine (Cytosar) 500 mg/m2 IV over 2 hours twice per day on days 1 to 5, should not be administered simultaneously with cladribine

35-day cycle for 2 or more cycles

Subsequent treatment

References

  1. Donadieu J, Bernard F, van Noesel M, Barkaoui M, Bardet O, Mura R, Arico M, Piguet C, Gandemer V, Armari Alla C, Clausen N, Jeziorski E, Lambilliote A, Weitzman S, Henter JI, Van Den Bos C; Salvage Group of the Histiocyte Society. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study. Blood. 2015 Sep 17;126(12):1415-23. Epub 2015 Jul 20. link to original article contains verified protocol link to PMC article PubMed

Vemurafenib monotherapy

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Regimen

Study Evidence
Hyman et al. 2015 Phase II, <20 pts in this arm

This was part of a basket trial, all patients had BRAF p.V600E mutations. A minority (7/18, 39%) of participants were untreated.

Chemotherapy

Duration of treatment not specified

References

  1. Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J. Vemurafenib in multiple nonmelanoma cancers with BRAF V600 mutations. N Engl J Med. 2015 Aug 20;373(8):726-736. link to original article contains verified protocol link to PMC article PubMed

Relapsed or refractory, consolidation

Cladribine monotherapy

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Regimen

Study Evidence
Donadieu et al. 2015 Phase II

Preceding treatment

Chemotherapy

21-day cycle for 2 cycles

Subsequent treatment

References

  1. Donadieu J, Bernard F, van Noesel M, Barkaoui M, Bardet O, Mura R, Arico M, Piguet C, Gandemer V, Armari Alla C, Clausen N, Jeziorski E, Lambilliote A, Weitzman S, Henter JI, Van Den Bos C; Salvage Group of the Histiocyte Society. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study. Blood. 2015 Sep 17;126(12):1415-23. Epub 2015 Jul 20. link to original article contains verified protocol link to PMC article PubMed

Relapsed or refractory, maintenance

Mercaptopurine, Methotrexate, Vinblastine, Prednisolone

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Regimen

Study Evidence
Donadieu et al. 2015 Phase II

Preceding treatment

Chemotherapy

Total of 18 months of maintenance

References

  1. Donadieu J, Bernard F, van Noesel M, Barkaoui M, Bardet O, Mura R, Arico M, Piguet C, Gandemer V, Armari Alla C, Clausen N, Jeziorski E, Lambilliote A, Weitzman S, Henter JI, Van Den Bos C; Salvage Group of the Histiocyte Society. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study. Blood. 2015 Sep 17;126(12):1415-23. Epub 2015 Jul 20. link to original article contains verified protocol link to PMC article PubMed