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

Pediatric Blood and Cancer

Volumn 60, Issue 2, 2013, Pages 175-184

  Haupt, Riccardo ^a   Minkov, Milen ^b   Astigarraga, Itziar ^c   Schäfer, Eva ^d   Nanduri, Vasanta ^e   Jubran, Rima ^f   Egeler, R Maarten ^g   Janka, Gritta ^h   Micic, Dragan ⁱ   Rodriguez Galindo, Carlos ^j   Van Gool, Stefaan ^k   Visser, Johannes ^l   Weitzman, Sheila ^g   Donadieu, Jean ^d  

^a G GASLINI INSTITUTE   (Italy)

^b ST ANNA CHILDREN S HOSPITAL   (Austria)

^c HOSPITAL DE CRUCES   (Spain)

^d ASSISTANCE PUBLIQUE HÔPITAUX DE PARIS   (France)

^e WATFORD GENERAL HOSPITAL   (United Kingdom)

^f CHILDREN S HOSPITAL LOS ANGELES   (United States)

^g HOSPITAL FOR SICK CHILDREN   (Canada)

^h UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

ⁱ Mother and Child Health Institute Belgrade rs   (Serbia)

^j BOSTON CHILDREN S HOSPITAL   (United States)

^k UNIVERSITY HOSPITALS LEUVEN   (Belgium)

^l UNIVERSITY HOSPITALS OF LEICESTER NHS TRUST   (United Kingdom)

more..

Author keywords

Clinical work up; Diagnosis; Follow up; Guidelines; Langerhans cell histiocytosis; Therapy

Indexed keywords

ANTINEOPLASTIC AGENT; AZATHIOPRINE; B RAF KINASE INHIBITOR; CHLORMETHINE; CLADRIBINE; CYTARABINE; EOSIN; ETOPOSIDE; HEMATOXYLIN; IMMUNOGLOBULIN; LANGERIN; MERCAPTOPURINE; METHOTREXATE; METHYLPREDNISOLONE; PREDNISONE; RETINOIC ACID; STEROID; T6 ANTIGEN; THALIDOMIDE; VINBLASTINE; VINCRISTINE;

ACUTE LYMPHOBLASTIC LEUKEMIA; AGE; ARTICLE; BLOOD CELL COUNT; BLOOD CHEMISTRY; BLOOD CLOTTING PARAMETERS; BONE DENSITY; BONE LESION; CARDIOPULMONARY ARREST; CEREBELLAR ATAXIA; CHOLESTEATOMA; CLINICAL EVALUATION; DEGENERATIVE DISEASE; DELAYED PUBERTY; DIABETES INSIPIDUS; DIFFERENTIAL DIAGNOSIS; DISEASE CLASSIFICATION; DRUG DOSE REDUCTION; ECZEMA; ELECTRON MICROSCOPY; EMPHYSEMA; ENDOCRINE DISEASE; ERYTHROCYTE SEDIMENTATION RATE; EVIDENCE BASED MEDICINE; FATIGUE; FOLLOW UP; GROWTH HORMONE DEFICIENCY; HEARING AID; HEMATOPOIETIC STEM CELL TRANSPLANTATION; HISTOLOGY; HUMAN; HYPOPITUITARISM; IMMOBILIZATION; IMMUNOPHENOTYPING; LANGERHANS CELL; LANGERHANS CELL HISTIOCYTOSIS; LEARNING DISORDER; LITERATURE; LOW DRUG DOSE; LUNG FIBROSIS; LUNG TRANSPLANTATION; LYMPHOMA; META ANALYSIS (TOPIC); MONOTHERAPY; MYELOID LEUKEMIA; NERVE DEGENERATION; NEUROTOXICITY; ORGAN SIZE; PAIN ASSESSMENT; PLASTIC SURGERY; PLEURECTOMY; PLEURODESIS; PNEUMOTHORAX; PRACTICE GUIDELINE; PRECOCIOUS PUBERTY; PRIORITY JOURNAL; PUBLICATION; PUVA; QUALITY OF LIFE; RADIOTHERAPY; RANDOMIZED CONTROLLED TRIAL (TOPIC); RESPIRATORY FAILURE; SCLEROSING CHOLANGITIS; SCOLIOSIS; SIDE EFFECT; SMOKING CESSATION; STAINING; SYSTEMATIC REVIEW (TOPIC); SYSTEMIC THERAPY; THORAX DRAINAGE; THORAX RADIOGRAPHY; ULTRASOUND;

ADOLESCENT; CHILD; CHILD, PRESCHOOL; HISTIOCYTOSIS, LANGERHANS-CELL; HUMANS;

EID: 84871126448     PISSN: 15455009     EISSN: 15455017     Source Type: Journal    
DOI: 10.1002/pbc.24367     Document Type: Article

References (60)

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.
2. Guyatt GH, Oxman AD, Sultan S, et al. GRADE guidelines: 9. Rating up the quality of evidence. J Clin Epidemiol 2011; 64: 1311-1316.
3. 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.
4. 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.
5. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues, 4th edition. Lyon: IARC press; 2008.
6. 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.
7. 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.
8. Gadner H, Grois N, Arico M, et al. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr 2001; 138: 728-734.
9. 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.
10. 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.
11. 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.
12. Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans' cell histiocytosis. Thorax 2000; 55: 405-416.
13. Vassallo R, Ryu JH, Colby TV, et al. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med 2000; 342: 1969-1978.
14. Braier J, Latella A, Balancini B, et al. Outcome in children with pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer 2004; 43: 765-769.
15. 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.
16. Grois N, Potschger U, Prosch H, et al. Risk factors for diabetes insipidus in Langerhans cell histiocytosis. Pediatr Blood Cancer 2006; 46: 228-233.
17. Haupt R, Nanduri VR, Egeler RM. Late effects of Langerhans cell histiocytosis and the association of LCH with malignancy. In: Egeler RM, Weitzman S, editor. Histiocytic disorders in children and adults. Cambridge, UK: Cambridge University Press; 2005.
18. Grois N, Fahrner B, Arceci RJ, et al. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr 2010; 156: 873-881, 881.
19. Berry DH, Gresik M, Maybee D, et al. Histiocytosis X in bone only. Med Pediatr Oncol 1990; 18: 292-294.
20. 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.
21. 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.
22. Dimentberg RA, Brown KL. Diagnostic evaluation of patients with histiocytosis X. J Pediatr Orthop 1990; 10: 733-741.
23. 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.
24. 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.
25. 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.
26. 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.
27. 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.
28. 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.
29. McClain KL, Kozinetz CA. A phase II trial using thalidomide for Langerhans cell histiocytosis. Pediatr Blood Cancer 2007; 48: 44-49.
30. 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.
31. 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.
32. Broadbent V, Pritchard J. Diabetes insipidus associated with Langerhans cell histiocytosis: Is it reversible? Med Pediatr Oncol 1997; 28: 289-293.
33. 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.
34. Rosenzweig KE, Arceci RJ, Tarbell NJ. Diabetes insipidus secondary to Langerhans' cell histiocytosis: Is radiation therapy indicated? Med Pediatr Oncol 1997; 29: 36-40.
35. 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.
36. 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.
37. 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.
38. 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.
39. 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.
40. 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.
41. 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.
42. 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.
43. 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.
44. 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.
45. 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.
46. 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.
47. 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.
48. 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.
49. 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.
50. 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.
51. 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.
52. 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.
53. Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 2010; 116: 1919-1923.
54. 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.
55. 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.
56. 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.
57. McClain K, Ramsay NK, Robison L, et al. Bone marrow involvement in histiocytosis X. Med Pediatr Oncol 1983; 11: 167-171.
58. Minkov M, Potschger U, Grois N, et al. Bone marrow assessment in Langerhans cell histiocytosis. Pediatr Blood Cancer 2007; 49: 694-698.
59. 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.
60. 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.