Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years

Riccardo Haupt, Milen Minkov, Itziar Astigarraga, Eva Schäfer, Vasanta Nanduri, Rima Jubran, R Maarten Egeler, Gritta Janka, Dragan Micic, Carlos Rodriguez-Galindo, Stefaan Van Gool, Johannes Visser, Sheila Weitzman, Jean Donadieu, Euro Histio Network, Riccardo Haupt, Milen Minkov, Itziar Astigarraga, Eva Schäfer, Vasanta Nanduri, Rima Jubran, R Maarten Egeler, Gritta Janka, Dragan Micic, Carlos Rodriguez-Galindo, Stefaan Van Gool, Johannes Visser, Sheila Weitzman, Jean Donadieu, Euro Histio Network

Abstract

These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented.

Copyright © 2012 Wiley Periodicals, Inc.

References

    1. Guyatt GH, Oxman AD, Vist GE, et al. GRADE: An emerging consensus on rating quality of evidence and strength of recommendations. BMJ. 2008;336:924–926.
    1. Guyatt GH, Oxman AD, Sultan S, et al. GRADE guidelines: 9. Rating up the quality of evidence. J Clin Epidemiol. 2011;64:1311–1316.
    1. Chikwava K, Jaffe R. Langerin (CD207) staining in normal pediatric tissues, reactive lymph nodes, and childhood histiocytic disorders. Pediatr Dev Pathol. 2004;7:607–614.
    1. Lau SK, Chu PG, Weiss LM. Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. Am J Surg Pathol. 2008;32:615–619.
    1. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th edition. Lyon: IARC press; 2008.
    1. Valladeau J, Ravel O, zutter-Dambuyant C, et al. Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules. Immunity. 2000;12:71–81.
    1. Jaffe R. The diagnostic histopathology of Langerhans cell histiocytosis. In: Weitzman S, Egeler M, editors. Histiocytic disorders of children and adults. Cambridge, UK: Cambridge University Press; 2005. pp. 14–39.
    1. Gadner H, Grois N, Arico M, et al. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr. 2001;138:728–734.
    1. Gadner H, Grois N, Potschger U, et al. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood. 2007;111:2556–2562.
    1. Minkov M, Grois N, Heitger A, et al. Response to initial treatment of multisystem Langerhans cell histiocytosis: An important prognostic indicator. Med Pediatr Oncol. 2002;39:581–585.
    1. Ronceray L, Potschger U, Janka G, et al. Pulmonary involvement in pediatric-onset multisystem langerhans cell histiocytosis: Effect on course and outcome. J Pediatr. 2012;161:129–133.
    1. Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans' cell histiocytosis. Thorax. 2000;55:405–416.
    1. Vassallo R, Ryu JH, Colby TV, et al. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med. 2000;342:1969–1978.
    1. Braier J, Latella A, Balancini B, et al. Outcome in children with pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer. 2004;43:765–769.
    1. Donadieu J, Rolon MA, Thomas C, et al. Endocrine involvement in pediatric-onset Langerhans' cell histiocytosis: A population-based study. J Pediatr. 2004;144:344–350.
    1. Grois N, Potschger U, Prosch H, et al. Risk factors for diabetes insipidus in Langerhans cell histiocytosis. Pediatr Blood Cancer. 2006;46:228–233.
    1. Haupt R, Nanduri VR, Egeler RM. Late effects of Langerhans cell histiocytosis and the association of LCH with malignancy. In: Egeler RM, Weitzman S, editors. Histiocytic disorders in children and adults. Cambridge, UK: Cambridge University Press; 2005.
    1. Grois N, Fahrner B, Arceci RJ, et al. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 2010;156:873–881. 881.
    1. Berry DH, Gresik M, Maybee D, et al. Histiocytosis X in bone only. Med Pediatr Oncol. 1990;18:292–294.
    1. Egeler RM, Thompson RC, Jr, Voute PA, et al. Intralesional infiltration of corticosteroids in localized Langerhans' cell histiocytosis. J Pediatr Orthop. 1992;12:811–814.
    1. Roger G, Dupre M, Leboulanger N, et al. Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: A complication amenable to curative surgery. Otol Neurotol. 2009;30:190–193.
    1. Dimentberg RA, Brown KL. Diagnostic evaluation of patients with histiocytosis X. J Pediatr Orthop. 1990;10:733–741.
    1. Raney RB, Jr, D'Angio GJ. Langerhans' cell histiocytosis (histiocytosis X): Experience at the Children's Hospital of Philadelphia, 1970–1984. Med Pediatr Oncol. 1989;17:20–28.
    1. Sessa S, Sommelet D, Lascombes P, et al. Treatment of Langerhans-cell histiocytosis in children. Experience at the Children's Hospital of Nancy. J Bone Joint Surg Am. 1994;76:1513–1525.
    1. Krafchik B, Pope E, Walsh SRA. Histiocytosis of the skin in children and adults. In: Weitzman S, Egeler M, editors. Histiocytic disorders of children and adults. Cambridge, UK: Cambridge University Press; 2005. pp. 130–153.
    1. Hadfield PJ, Birchall MA, Albert DM. Otitis externa in Langerhans' cell histiocytosis—The successful use of topical nitrogen mustard. Int J Pediatr Otorhinolaryngol. 1994;30:143–149.
    1. Steen AE, Steen KH, Bauer R, et al. Successful treatment of cutaneous Langerhans cell histiocytosis with low-dose methotrexate. Br J Dermatol. 2001;145:137–140.
    1. Womer RB, Anunciato KR, Chehrenama M. Oral methotrexate and alternate-day prednisone for low-risk Langerhans cell histiocytosis. Med Pediatr Oncol. 1995;25:70–73.
    1. McClain KL, Kozinetz CA. A phase II trial using thalidomide for Langerhans cell histiocytosis. Pediatr Blood Cancer. 2007;48:44–49.
    1. Donadieu J, Egeler M, Pritchard J. Langerhans cell histiocytosis: A clinical update. In: Weitzman S, Egeler M, editors. Histiocytic disorders of children and adults. Cambridge, UK: Cambridge University Press; 2005. pp. 95–129.
    1. Ottaviano F, Finlay JL. Diabetes insipidus and Langerhans cell histiocytosis: A case report of reversibility with 2-chlorodeoxyadenosine. J Pediatr Hematol Oncol. 2003;25:575–577.
    1. Broadbent V, Pritchard J. Diabetes insipidus associated with Langerhans cell histiocytosis: Is it reversible? Med Pediatr Oncol. 1997;28:289–293.
    1. Minehan KJ, Chen MG, Zimmerman D, et al. Radiation therapy for diabetes insipidus caused by Langerhans cell histiocytosis. Int J Radiat Oncol Biol Phys. 1992;23:519–524.
    1. Rosenzweig KE, Arceci RJ, Tarbell NJ. Diabetes insipidus secondary to Langerhans' cell histiocytosis: Is radiation therapy indicated? Med Pediatr Oncol. 1997;29:36–40.
    1. Ng Wing TS, Martin-Duverneuil N, Idbaih A, et al. Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: A nation wide retrospective study. Orphanet J Rare Dis. 2011;6:83.
    1. Dhall G, Finlay JL, Dunkel IJ, et al. Analysis of outcome for patients with mass lesions of the central nervous system due to Langerhans cell histiocytosis treated with 2-chlorodeoxyadenosine. Pediatr Blood Cancer. 2008;50:72–79.
    1. Donadieu J, Piguet C, Bernard F, et al. A new clinical score for disease activity in Langerhans cell histiocytosis. Pediatr Blood Cancer. 2004;43:770–776.
    1. The French Langerhans' Cell Histiocytosis Study Group. A multicentre retrospective survey of Langerhans' cell histiocytosis : 348 cases observed between 1983 and 1993. Arch Dis Child. 1996;75:17–24.
    1. Bernard F, Thomas C, Bertrand Y, et al. Multi-centre pilot study of 2-chlorodeoxyadenosine and cytosine arabinoside combined chemotherapy in refractory Langerhans cell histiocytosis with haematological dysfunction. Eur J Cancer. 2005;41:2682–2689.
    1. Steiner M, Matthes-Martin S, Attarbaschi A, et al. Improved outcome of treatment-resistant high-risk Langerhans cell histiocytosis after allogeneic stem cell transplantation with reduced-intensity conditioning. Bone Marrow Transplant. 2005;36:215–225.
    1. Weitzman S, Braier J, Donadieu J, et al. 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;53:1271–1276.
    1. Idbaih A, Donadieu J, Barthez MA, et al. Retinoic acid therapy in “degenerative-like” neuro-Langerhans cell histiocytosis: A prospective pilot study. Pediatr Blood Cancer. 2004;43:55–58.
    1. Allen CE, Flores R, Rauch R, et al. Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside. Pediatr Blood Cancer. 2009;54:416–423.
    1. Gavhed D, Laurencikas E, Akefeldt SO, et al. Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis. Acta Paediatr. 2011;100:e36–e39.
    1. Buchler T, Cervinek L, Belohlavek O, et al. Langerhans cell histiocytosis with central nervous system involvement: Follow-up by FDG-PET during treatment with cladribine. Pediatr Blood Cancer. 2005;44:286–288.
    1. Haupt R, Nanduri V, Calevo MG, et al. Permanent consequences in Langerhans cell histiocytosis patients: A pilot study from the Histiocyte Society-Late Effects Study Group. Pediatr Blood Cancer. 2004;42:438–444.
    1. Nanduri VR, Pritchard J, Levitt G, et al. Long term morbidity and health related quality of life after multi-system Langerhans cell histiocytosis. Eur J Cancer. 2006;42:2563–2569.
    1. GH Research Society. Consensus guidelines for the diagnosis and treatment of growth hormone (GH) deficiency in childhood and adolescence: Summary statement of the GH Research Society. GH Research Society. J Clin Endocrinol Metab. 2000;85:3990–3993.
    1. Schmahmann JD, Gardner R, MacMore J, et al. Development of a brief ataxia rating scale (BARS) based on a modified form of the ICARS. Mov Disord. 2009;24:1820–1828.
    1. Trouillas P, Takayanagi T, Hallett M, et al. International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. J Neurol Sci. 1997;145:205–211.
    1. Curry HL, Parkes SE, Powell JE, et al. Caring for survivors of childhood cancers: The size of the problem. Eur J Cancer. 2006;42:501–508.
    1. Kenney LB, Bradeen H, Kadan-Lottick NS, et al. The current status of follow-up services for childhood cancer survivors, are we meeting goals and expectations: A report from the Consortium for New England Childhood Cancer Survivors. Pediatr Blood Cancer. 2011;57:1062–1066.
    1. Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116:1919–1923.
    1. Satoh T, Smith A, Sarde A, et al. B-RAF mutant alleles associated with Langerhans cell histiocytosis, a granulomatous pediatric disease. PLoS ONE. 2012;7:e33891.
    1. Haroche J, Charlotte F, Arnaud L, et al. High prevalence of BRAF V600E mutations in Erdheim–Chester disease but not in other non-Langerhans cell histiocytoses. Blood. 2012;120:2700–2703.
    1. Phillips M, Allen C, Gerson P, et al. Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatr Blood Cancer. 2009;52:97–101.
    1. McClain K, Ramsay NK, Robison L, et al. Bone marrow involvement in histiocytosis X. Med Pediatr Oncol. 1983;11:167–171.
    1. Minkov M, Potschger U, Grois N, et al. Bone marrow assessment in Langerhans cell histiocytosis. Pediatr Blood Cancer. 2007;49:694–698.
    1. Favara BE, Jaffe R, Egeler RM. Macrophage activation and hemophagocytic syndrome in Langerhans cell histiocytosis: Report of 30 cases. Pediatr Dev Pathol. 2002;5:130–140.
    1. Galluzzo ML, Braier J, Rosenzweig SD, et al. Bone marrow findings at diagnosis in patients with multisystem Langerhans cell histiocytosis. Pediatr Dev Pathol. 2010;13:101–106.

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