Langerhans cell histiocytosis in children and adolescents

Česká verzia

Authors: H. Mottl
Authors place of work: Klinika dětské hematologie a onkologie 2. lékařské fakulty UK a FN Motol Praha, přednosta prof. MU Dr. Jan Starý, DrSc.
Published in the journal: Vnitř Lék 2010; 56(Supplementum 2): 64-73
Category: Histiocytóza z Langerhansových buněk a některá další vzácná hematologická onemocnění

Summary

The histiocytosis represents the rare diseases with large biological behavior and clinical symptoms, as well. The Langerhans cell histiocytosis is the most frequent entities in children in this category. The paper does the summary of the etiology, biology, clinical aspects, diagnostic possibilities, and therapeutic outcomes of LCH. The crucial prognostic value represents disease extension: single-system in compare with multi-system LCH. The role of treatment in some LCH forms is questinable but in other forms the chemotherapy is important. Histiocytosis affects all age groups but more than 50% cases concern children up to 15 years of age. There are not gender predominance but exist age dependent predisposition (e. g. bone disease in children 5– 15 years of age or multi-system disease in children less than 2 years of age). Multi-system LCH incidence is aproximately 2.6 children/ 1 milion children/ year (9 in chidren less to 1 year of age and 0.7 in age group 10– 14 years). In the Czech Republic supposed 10– 20 children per year. Histiocytosis characterized abnormal accumulation mononuclear phagocytic cells (dendritic cells and macrophages) which are derivate from primary CD34⁺ hematopoietic stem cells. The basic point in the differentiation process has large scope of cytokines. LCH was considered as malignant disease, reactive or aberrant immunological answer of organism in the previous time. The last review showed disease with pathological Langerhans cells in granulomatous lesion together with common inflamantory elements as eosinophils, lymphocytes and macrophages. The cell infiltration and clinical symptoms are explained as outcome of aberant secretion these cells and activated T-lymphocytes. Therefore clinical symptoms showed different type of manifestation from spontaneous regression through recurrent behavior to malignant multi-system disease with fatal outcome. Diagnosis is done by histopathological investigation with proof of intracellular Birbecks granules, imunohistochemical S-100 protein positivity, and CD1a⁺ as well. But the Langerhans cell histiocytosis etiology and pathogenesis in unknown so far. The treatment strategy depends on the spread of disease.

Key words:
Langerhans cell histiocytosis – children – adolescents

Zdroje

1. Adam Z, Kavan P, Koutecký J. et al. Histiocytóza z Langerhansových buněk. Čs Pediatr 1997; 52: 899– 905.

2. Arceci RJ, Brenner MK, Pritchard J. Controversies and new approaches to treatment of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12: 339– 357.

3. Aricò M, Egeler RM. Clinical aspects of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12: 247– 258.

4. Avery ME, McAfee JG, Guild HG. The course and prognosis of reticuloendotheliosis (eosinophilic granuloma, Schüller- Christian disease and Letterer- Siwe disease): a study of forty cases. Am J Med 1957; 22: 636– 652.

5. Bank MI, Rengtved P, Carstensen H et al. Langerhans cell histiocytosis: an evaluation of histopathological parameters, demonstration of proliferation by Ki- 67 and mitotic bodies. APMIS 2003; 111: 300– 308.

6. Bechan GI, Egeler RM, Arceci RJ. Biology of Langerhans cells and Langerhans cell histiocytosis. Int Rev Cytol 2006; 254: 1– 43.

7. 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.

8. Bernstrand C, Carstensen H, Jakobsen B et al. Immunogenetic heterogeneity in single-system and multi-system langerhans cell histiocytosis. Pediatr Res 2003; 54: 30– 36.

9. Berry DH, Gresik M, Maybee D et al. Histiocytosis X in bone only. Med Pediatr Oncol 1990; 18: 292– 294.

10. Bhatia S, Nesbit ME Jr, Egeler RM et al. Epidemiologic study of Langerhans cell histiocytosis in children. J Pediatr 1997; 130: 774– 784.

11. Binkovitz LA, Olshefski RS, Adler BH. Coincidence FDG- PET in the evaluation of Langerhans’ cell histiocytosis: preliminary findings. Pediatr Radiol 1997; 33: 598– 602.

12. Braier J, Ciocca M, Latella A et al. Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell histiocytosis. Med Pediatr Oncol 2002; 38: 178– 182.

13. Brauner MW, Grenier P, Tijani K et al. Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans. Radiology 1997; 204: 497– 502.

14. Broadbent V, Gadner H. Current therapy for Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12: 327– 338.

15. Carstensen H, Ornvold K. The epidemiology of Langerhans cell histiocytosis in children in Denmark. Med Pediatr Oncol 1993; 21: 387– 388.

16. Christian HA. Defects in membranous bones, exophthalmos and diabetes insipidus: an unusual syndrome of dyspituitarism. Med Clin North Am 1920; 3: 849– 871.

17. Coppes- Zantinga A, Egeler RM. Historical review the 9. Langerhans cell histiocytosis X fines revealed. Br J Haematol 2002; 16: 3– 9.

18. Dacic S, Trusky C, Bakker A et al. Genotypic analysis of pulmonary Langergans cell histiocytosis. Hum Pathol 2007; 34: 1345– 1349.

19. de Graaf JH, Tamminga RY, Dam- Meiring A et al. The presence of cytokines in Langerhans’ cell histiocytosis. J Pathol 1996; 180: 400– 406.

20. Dogan AS, Conway JJ, Miller JH et al. Detection of bone lesions in Langerhans cell histiocytosis: complementary roles of scintigraphy and conventional radiography. J Pediatr Hematol Oncol 1996; 18: 51– 58.

21. Donadieu J, Rolon MA, Pion I et al. Incidence of growth hormone deficiency in pediatric- onset Langerhans cell histiocytosis: efficacy and safety of growth hormone treatment. J Clin Endocrinol Metab 2004; 89: 604– 609.

22. 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.

23. Donadieu J, Egeler RM, Pritchard J. Langerhans cell histiocytosis: a clinical update. In: Weitzman S, Egeler RM (ed). Histiocytic disorders of children and adults. Cambridge: Cambridge University Press 2005: 5– 129.

24. De Graaf JH, Tamminga RY, Dam- Meiring A et al. The presence of cytokines in Langerhans’ cell histiocytosis. J Pathol 1996; 180: 400– 406.

25. Egeler RM, Thompson RC Jr, Voûte PA et al. Intralesional infiltration of corticosteroids in localized Langerhans’ cell histiocytosis. J Pediatr Orthop 1992; 12: 811– 814.

26. Egeler RM, D’Angio GJ. Langerhans cell histiocytosis. J Pediatr 1995; 127: 1– 11.

27. Egeler RM, Neglia JP, Aricò M et al. The relation of Langergans cell histiocytosis to acute leukemia, lyphomas, and other solid tumors. The LCH- Malignancy Study Group of the Hisitocyte Society. Hematol Oncol Clin North Am 1998; 12: 369– 378.

28. Egeler RM, Favara BE, van Meurs M et al. Differential In situ cytokine profiles of Langerhans-like cells and T cells in Langerhans cell histiocytosis: abundant expression of cytokines relevant to disease and treatment. Blood 1999; 94: 4195– 4201.

29. Farber S. The nature of solitary or eosinophilic granuloma’ of bone. Am J Pathol 1941; 17: 625– 629.

30. Farran RP, Zaretski E, Egeler RM. Treatment of Langerhans cell histiocytosis with pamidronate. J Pediatr Hematol Oncol 2001; 23: 54– 56.

31. Favara BE, McCarthy RC, Mierau GW. Histiotosis X. Hum Pathol 1983; 14: 663– 676.

32. 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.

33. Gadner H, Grois N. The histiocytosis syndromes. In: Fitzpatrick TB, Eisen AZ, Wolff K et al (eds). Dermatology in general medicine. Vol. II. 4th ed. New York: McGraw- Hill, Inc. 1993: 2003– 2017.

34. Gadner H, Heitger A, Grois N et al. Treatment strategy for disseminated Langerhans cell histiocytosis. DAL HX- 83 Study Group. Med Pediatr Oncol 1994; 23: 72– 80.

35. Gadner H, Ladish S. The treatment of Langerhans cell histiocytosis. In: Weitzman S, Egeler RM (eds). Histiocytic disorders of children and adults. Cambridge: Cambridge University Press 2005: 229– 253.

36. Glotzbecker MP, Carpentieri DF, Dormans JP. Langerhans cell histiocytosis: a primary viral infection of bone? Human herpes virus 6 latent protein detected in lymphocytes from tissue of children. J Pediatr Orthop 2004; 24: 123– 129.

37. Goo HW, Yang DH, Ra YS et al. Whole- body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol 2006; 36:1019– 1031.

38. Gramatovici R, D’Angio GJ. Radiation therapy in soft- tissue lesions in histiocytosis X (Langerhans’ cell histiocytosis). Med Pediatr Oncol 1988; 16: 259– 262.

39. Greenberger JS, Cassady JR, Jaffe N et al. Radiation therapy in patients with histiocytosis: management of diabetes insipidus and bone lesions. Int J Radiat Oncol Biol Phys 1979; 5: 1749– 1755.

40. Grois NG, Favara BE, Mostbeck GH et al. Central nervous system disease in Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12: 287– 305.

41. Grois N, Prayer D, Prosch H et al. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 2005; 128: 829– 838.

42. Hand A. Polyuria and tuberculosis. Arch Pediatr 1893; 10: 673– 675.

43. 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.

44. Howarth DM, Mullan BP, Wiseman GA et al. Bone scintigraphy evaluated in diagnosing and staging Langerhans’ cell histiocytosis and related disorders. J Nucl Med 1996; 37: 1456– 1460.

45. Jaffe R. Patology of histiocytosis X. Perspect Pediatr Pathol 1987; 9: 4– 47.

46. Jaffe ES, Harris NL, Stein H et al. World Health Organization classification of tumours. Pathology and genetics. Tumors of Haematopoietic and lymphoid tissues. Lyon: IARC Press 2001: 32– 41.

47. Jenson HB, McClain KL, Leach CT et al. Evaluation of human herpesvirus type 8 infection in childhood Langerhans cell histiocytosis. Am J Hematol 2000; 64: 237– 241.

48. Kakkar S, Kapila K, Verma K. Langerhans cell histiocytosis in lymph nodes. Cytomorphologic diagnosis and pitfalls. Acta Cytol 2001; 45: 327– 332.

49. Karis J, Bernstrand C, Fadeel B et al. The incidence of Langerhans cell histiocytosis in children in Stockholm County, Sweden 1992– 2001. Proceedings of the XIX Meeting of Histiocyte Society. Philadephia 2003.

50. Kawakubo Y, Kishimoto H, Sato Y et al. Human cytomegalovirus infection in foci of Langerhans cell histocytosis. Virchows Arch 1999; 434: 109– 115.

51. Kilpatrick SE, Wenger DE, Gilchrist GS et al. Langerhans’ cell histiocytosis (histiocytosis X) of bone. A clinicopathologic analysis of 263 pediatric and adult cases. Cancer 1995; 76: 2471– 2484.

52. Ladish S, Gadner H. Treatment of Langerhans cell histiocytosis – evolution and current approaches. Br J Cancer Suppl 1994; 23: S41– S46.

53. Laman JD, Leenen PJ, Annels NE et al. Langerhans- cell histiocytosis ‘insight into DC biology’. Trends Immunol 2003; 24: 190– 196.

54. Lampert F. Langerhans cell histiocytosis. Historical perspectives. Hematol Oncol Clin North Am 1998; 12: 213– 219.

55. Lau L, Krafchik B, Trebo MM et al. Cutaneous Langerhans cell histiocytosis in children under one year. Pediatr Blood Cancer 2006; 46: 66– 71.

56. Le Deley MC, Vassal G, Taïbi A et al. High cumulative rate of secondary leukemia after continuous etoposide treatment for solid tumors in children and young adults. Pediatr Blood Cancer 2005; 45: 25– 31.

57. Letterer E. Aleukamische retikulose (ein Beitrag zu den proliferativen Erkrankungen des reticuloendothelialapparates). Frankfurter Zeitschritte Pathol 1924; 30: 377– 394.

58. LCH Study Group of the Histiocyte Society. LCH II – Treatment Protocol of the Second International Study for Langerhans cell histiocytosis. Protocol 1996.

59. LCH Study Group of the Histiocyte Society. LCH III – Treatment Protocol of the Second International Study for Langerhans cell histiocytosis. Protocol 2001.

60. Lichtenstein L. Hisitocytosis X: integration of eosinophilic granuloma of bone, Letterer- Siwe disease and Schüller- Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 1953; 56: 84– 102.

61. Lopes LF, de Camargo B. Secondary acute promyelocytic leukemia after treatment with etoposide for Langerhans cell histiocytosis (LCH). Med Pediatr Oncol 1999; 32: 315.

62. McClain KL, Gonzales J, Jonkers R et al. Need for a cooperative study: pulmonary Langerhans cell histiocytosis and its management in adults. Med Pediatr Oncol 2002; 39: 35– 39.

63. McClain KL, Laud P, Wu WS et al. Langerhans cell histiocytosis patients have HLA Cw7 and DR4 types associated with specific clinical presentations and no increased frequency in polymorphism of the tumor necrosis factor alpha promoter. Med Pediatr Oncol 2003; 41: 502– 507.

64. Minkov M, Grois N, Heitger A et al. Treatment of multisystem Langerhans cell histiocytosis. Results of the DAL- HX 83 and DAL- HX 90 studies. DAL- HX Study Group. Klin Pediatr 2000; 212: 139– 144.

65. Minkov M, Grois N, Heitger A et al. DAL- HX Study Group. Response to initial treatment of multisystem Langerhans cell histiocytosis: an important prognostic indicator. Med Pediatr Oncol 2002; 39: 581– 585.

66. Mottl H, Mráček J, Kabelka Z et al. Hisitiocytóza z Langerhansových buněk u děti. Čs Pediatr 1992; 47: 530– 533.

67. Mottl H, Ganevová M, Radvanská J et al. Léčebné výsledky histiocytózy z Langerhansových buněk protokolem LCH II. Čas Lék Čes 2005; 144: 753– 755.

68. Mottl H, Nekolná M, Starý J et al. Plicní forma histiocytózy z Langerhansových buněk u dětí. 15. Konference dětských hematologů a onkologů ČR a SR. České Budějovice, Abstrakta 2005: 14.

69. Mottl H, Starý J, Šmelhaus V et al. Současná strategie léčby histiocytózy z Langerhansových buněk u dětí. XXX. brněnské onkologické dny 2006: 140.

70. Mottl H, Stary J, Snajdauf J et al. Pulmonary Langerhans cell histiocytosis in children and adolescents. Pediatr Blood Cancer 2006; 47: 451.

71. Mottl H, Starý J. Histiocytóza z Langerhansových buněk u dětí – klinické projevy, diagnostika a současná léčba. Čs Pediatr 2007; 62: 220– 225.

72. Mottl H, Stary J, Snajdauf J et al. Primary pulmonary Langerhans cell histiocytosis: Outcome in children and adolescents. 23rd Annual Meeting of Histiocyte Society, 23. 9.– 25 .9. 2007. Cambridge, UK. Abstract.

73. Munn SE, Olliver L, Broadbent V et al. Use of indomethacin in Langerhans cell histiocytosis. Med Pediatr Oncol 1999; 32: 247– 249.

74. Nanduri VR, Kelly K, Malone M et al. Colon involvement in Langerhans’ cell histiocytosis. J Pediatr Gastroenterol Nutr 1999; 29: 462– 466.

75. Neumann C, Schaumberg- Lever G, Döpfer R et al. Interferon gamma is a marker for histiocytosis X cells in the skin. J Invest Dermatol 1988; 91: 280– 282.

76. Nezelof C. Histiocytosis X: a histological and histogenetic study. Perspect Pediatr Pathol 1979; 5: 153– 178.

77. Osband ME, Lipton JM, Lavin P et al. Histiocytosis- X. N Engl J Med 1981; 304: 146– 153.

78. Prayer D, Grois N, Prosch H et al. MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 2004; 25:880– 891.

79. Pritchard J, Broadbent V. Histiocytosis – an introduction. Br J Cancer Suppl 1994; 23: S1– S3.

80. Schmitz L, Favara BE. Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12: 221– 246.

81. Schouten B, Egeler RM, Leenen PJ et al. Expression of cell cycle-related gene products in Langerhans cell histiocytosis. J Pediatr Hematol Oncol 2002; 24: 727– 732.

82. Schüller A. Uber eigenartige schadeldefekte im jugendalter. Fortschritte Auf der Gebiete Rontgenstrahlen 1915; 23: 12– 18.

83. Selch MT, Parker RG. Radiation therapy in management of Langerhans cell histiocytosis. Med Pediatr Oncol 1990; 18: 97– 102.

84. Siwe SA. Die Reticuloendotheliose-ein neues krankheitsbild unter den hepatosplenomegalien. Zeitschrift für Kinderheilkunde 1933; 55: 212– 247.

85. Slacmeulder M, Geissmann F, Lepelletier Y. No association between Langerhans cell histiocytosis and human herpes virus- 8. Med Pediatr Oncol 2002; 39: 187– 189.

86. Slater JM, Swarm OJ. Eosinophilic granuloma of bone. Med Pediatr Oncol 1980; 8:151– 164.

87. 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.

88. Stine KC, Saylors RL, Saccente S et al. Efficacy of continuous infusion 2- CDA (cladribine) in pediatric patients with Langerhans cell histiocytosis. Pediatr Blood Cancer 2004; 43: 81– 84.

89. Stuurman KE, Lau I, Doda W et al. Evaluation of the natural history and long term complications of patients with Langerhans cell histiocytosis of bone. Proc XIX meeting Histiocyte Society. Philadelphia 2003.

90. Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans’ cell histiocytosis. Thorax 2002; 55: 405– 416.

91. Titgemeyer C, Grois N, Minkov M et al. Pattern and course of single-system disease in Langerhans cell histiocytosis data from the DAL- HX 83- and 90- study. Med Pediatr Oncol 2001; 37: 108– 114.

92. Vassallo R, Ryu JH, Colby TV et al. Pulmonary Langerhans’- cell histiocytosis. N Engl J Med 2000; 342: 1969– 1978.

93. Weitzman S, Egeler RM. Histiocytic disorders of children and adults: introduction to the problem, overview, historical perspective and epidemiology. In: Weitzman S, Egeler RM (eds). Histiocytic disorders of children and adults. Cambridge: Cambridge University Press 2005: 1– 13.

94. Willis B, Ablin A, Weinberg V et al. Disease course and late sequelae of Langerhans’ cell histiocytosis: a 25 year experience at the University of California, San Francisco. J Clin Oncol 1996; 14: 2073– 2082.

95. Willman CL, Busque L, Griffith BB et al. Langehans’ cell histiocytosis (histiocytosis X) a clonal proliferative disease. N Engl J Med 1994; 331: 154– 160.

96. Womer RB, Anunciato KR, Chehrenama M. Oral methotrexate and alternace- day prednisone for low- risk Langerhans cell histiocytosis. Med Pediatr Oncol 1995; 25: 70– 73.

97. Wong A, Ortiz- Neira CL, Reslan WA et al. Liver involvement in Langerhans cell histiocytosis. Pediatr Radiol 2006; 36: 1105– 1107.

98. Woo KI, Harris GJ. Eosinophilic granuloma of the orbit: understanding the paradox of aggressive destruction responsive to minimal intervention. Ophthal Plast Reconstr Surg 2003; 19: 429– 439.

99. Writing Group of the Histiocyte Siociety. Histiocytosis syndromes in children. Lancet 1987; 1: 208– 209.

100. Yousem SA, Colby TV, Chen YY et al. Pulmonary Langerhans’- cell histiocytosis. Molecular analysis of clonality. Am J Surg Pathol 2001; 25: 630– 636.

101. Yu RC, Chu C, Buluwela L et al. Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis. Lancet 1994; 343: 767– 768.

102. Zouny JW. Cell fate development in the myeloid system. In: Lotze MT, Thomson AW (eds). Dendritic cells. San Diego: Academic Press 1999: 29– 49.