Histiocytosis from Langerhans cell in adults – experience of one department and review of treatment alternatives
Authors:
Z. Adam 1; J. Vaníček 2; P. Šlampa 3; P. Čoupek 3; I. Mareschova 1; J. Neubauer 4; L. Babičková 5; Z. Adamová 6; M. Tomíška 1; M. Navrátil 1
Authors‘ workplace:
Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.
1; Klinika zobrazovacích metod Lékařské fakulty MU a FN u sv. Anny, Brno, přednosta doc. MUDr. Petr Krupa, CSc.
2; Radioterapeutické oddělení Masarykova onkologického ústavu, Brno, přednosta doc. MUDr. Pavel Šlapa, CSc.
3; Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil Válek, CSc.
4; Klinika tuberkulózy a respiračních nemocní Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednostka doc. MUDr. Jana Skřičková, CSc.
5; Zdavotní středisko pro děti a dorost, Obilní trh, Brno, vedoucí MUDr. Zdenka Adamová
6
Published in:
Vnitř Lék 2006; 52(4): 355-370
Category:
Original Contributions
Overview
Thirteen patients with Langerhans cell histiocytosis (LCH) have been treated in hospital Brno Masaryk University during the last 15 years. In 4 cases of this total amount, the diagnosis was made in childhood and these young adults were referred to our department from Pediatric cancer center. In 9 cases, the diagnosis has been made in people elder than 18 years. The disease recidived in 3 patients with LCH diagnosed in childhood, in one case with neurodegenerative impairment of brain. In 4 patients from the total of 9 with LCH diagnosed in age over 18 years, the disease had aggressive course with several recidives. In one case it was pulmonal and multifocal osseal manifestation, in two cases multifocal osseal disease. Isolated pulmonal form of LCH was diagnosed only in one patient. By the first patient, high dose melphalan and etoposide with peripheral blood stem cell transplantation was performed after failure of vincristin and prednison therapy and failure of etoposide therapy. The first remission after this high dose therapy lasted only 2.5 years. For relapse second cycle of the same high dose therapy was administered, but the next remission was much shorter. By the two patients with multifocal recidives in bones 2-chlordeoxyadenosine was administered as initial therapy. These patients are followed up for more then 24 months and they are without relapse of this disease. The 2-chlordeoxyadenosine is very efficient in multifocal bone form of LCH and has potential to reach long remission. Therefore in a case of aggressive multifocal disease we would prefer 2-chlordeoxyadenosine therapy as therapy of the first choice.
Key words:
Langerhans cell histiocytosis - 2-chlordeoxyadenosine
Sources
1. Akkari V, Donadieu J, Piguet C et al. Hematopoietic stem cell transplantation in patients with severe Langerhans cell histiocytosis and hematological dysfunction: experience of the French Langerhans Cell Study Group. Bone Marrow Transplant 2003; 31: 1097-1103.
2. Anton M, Holoušova M, Řehůřek J et al. Histiocytoza X a dětská očnice. Čs Ophthal 1992; 48: 176-180.
3. Arceci RJ, Brenner MK, Pritchard J et al. Contraversis and new approaches to treatment of Langerhans cell histiocytosis. Hematol Oncol Clin North Amer 1998; 12: 339-357.
4. Arico M, Colella R, Conter V et al. Cyclosporine therapy for refractory Langerhans cell histiocytosis. Med Pediatr Oncol 1995; 25: 12-16.
5. Arzoo K, Sadeghi S, Pullarkar V. Pamidronate for bone pain from osteolytic lesions in Langerhans cell histiocytosis. N Engl J Med 2001; 345: 225-226.
6. Baumgartner I, Hochstetter A, Baumert B et al. Langerhans cell histiocytosis in adults. Med Pediatric Oncol 1997; 28: 9-14.
7. Bellmunt J, Albanell J, Salud A et al. Interferon and disseminated Langerhans cell histiocytosis. Med Pediatr Oncol 1992; 20: 336-337.
8. 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.
9. Bernstrand C, Björk O, Ahnström L et al. Intralesional steroids in Langerhans cell histiocytosis of bone. Acta Paediatr 1996; 85: 502-504.
10. Bernstrand C, Cederlund K, Sandstedt B et al. Pulmonary abnormalities at long term follow up of patients with Langerhans cell histiocytosis. Med Pediatr Oncol 2001; 36: 459-468.
11. Berry J, Russel JA. Salvage of relapsed malignant histiocytosis by autologous bone marrow transplant. Bone Marrow Transplant 1989; 4: 123-124.
12. Bertolini F, Mingrone W, Alietti A et al. Thalidomide in multiple myeloma, myelodysplastic syndromes and histiocytosis. Ann Oncol 2001; 12: 987-990.
13. Bittenglova R, Pešek M, Mukenšnabl P et al. Granulomatóza z Langerhansových buněk. Stud Pneumol Phtiseol 2002; 62: 196-202.
14. Blum R, Seymour JF, Hicks RJ. Role of FDG-positron emission tomography scannin in the management of histiocytosis. Leukem Lymphoma 2002; 43: 2155-2157.
15. Broadbent V, Gadner H. Current therapy for Langerhans cell histiocytosis. Hematol Oncol Clin North Amer 1998; 12: 327-336.
16. Broadbent V, Pritchard J, Yeomans E. Etoposide in the treatment of multisystem Langerhans cell hitocytosis. Med Pediatr Oncol 1989; 17: 97-100.
17. Brown RE. Interpheron alpha therapy protein kinse C alpha and Langerhans cell histiocytosis. Med Pediatr Oncol 2003; 41: 63-64.
18. Brown RE. Bisphosphonates as antialveolar macrophage therapy in pulmonary Langerhans cell histiocytosis. Med Pediatr Oncol 2001; 36: 641-643.
19. Bunanska E, Stančokova T, Dluholucky S. Histiocytóza z Langerhansových buněk. Čes Slov Pediat 1998; 53: 18-19.
20. Cassady JR. Current role of radiation therapy in the management of histiocytosis X. Hematol Oncol N Am 1987; 1: 123-125.
21. Choi SW, Bangaru BS, Wu CD et al. Gastrointestinal involvement in disseminated Langerhans cell histiocytosis (LCH) with durable complete response to 2-chlorodeoxyadenosine and high-dose cytarabine. J Pediatr Hematol Oncol 2003; 25: 503-506.
22. Conter V, Reciputo A, Arrigo C et al. Bone marrow transplantation for refractory Langerhans' cell histiocytosis. Haematologica 1996; 81: 468-471.
23. Culic S, Jakobson A, Culic V. Etoposide a the basis and interferon alfa as the maitenance therapy for Langerhans cell histiocytosis. Pediatr Hematol Oncol 2001; 18: 291-294.
24. Dallafior S, Pugin P, Cerny T et al. Successful treatment of a case of cutaneous Langerhans cell granulomatosis with 2-chlorodeoxyadenosine and thalidomide. Hautarzt 1995; 46: 553-560.
25. Dina A, Zahava V, Iness M. The role of vascular endothelial growth factor in Langerhans cell histiocytosis. J Pediatr Hematol Oncol 2005; 27: 62-66.
26. Dogan S, Conway J, Miller JH et al. Detection of bone lesions in Langerhans cell histiocytosis. Complementary roles of scintigraphy and conventional radiograpy. J Pediatric Hematol/Oncol 1996; 18: 51-58.
27. Donadieu J, Rolon MA, Pion I et al. Incidence of growth hormone difficienci pediatric onset Langerhans cell histiocytosis: Efficacy and safety of Growth hormone treatment. J Clin Endokrinol Metabolism 2004; 89: 604-609.
28. Dornfeld S, Winkler C, Dörr W et al. Stralentherapie der Histiocytosis X. Stralenther. Onkologie 1998; 174: 534-535.
29. Egeler MR, de Kraker J, Voute PA. Cytosine-arabinoside, vincristine and prednisolone in the treatment of children with disseminated Langerhans cell histiocytosis with organs dysfunction. Med Pediatr Oncol 1993; 20: 265-270.
30. Egeler MR, Thompson RC, Voute PA et al. Intralesional infiltration of corticosteroids in localized Langerhans cell histiocytosis. J Pediatr Orthop 1992; 12: 811-814.
31. Egeler RM. More on pamidronate in Langerhans cell histiocytosis. N Engl J Med 2001; 345: 1502-1503.
32. Elomaa I, Blomquist C, Porkka L et al. Experience of clodronate treatment of multifocal eosinophilic granuloma of bone. J Intern Med 1989; 225: 59-61.
33. Farran RP, Zaretski E, Egeler RM. Treatment of Langerhans cell histiocytosis with pamidronate. J Pediatr Hematol Oncol 2001; 23: 54-56.
34. Favara BE, Feller A and members WHO committee on Histiocyte/Retikulum Cell Proliferations. Contemporary classification of histiocyte disorders. Medical Pediatric Onkol 1997; 29: 157-166.
35. Ferreli C, Aste N, Pinna LA et al. Langerhans cell histiocytosis in an adult. J Eur Acad Dermatol Venereol 1997; 9: 253-255.
36. Gadner H, Heitger A, Grois N et al. Treatment strategy for disseminated Langerhans cell histiocytosis. Med Pediatr Oncol 1994; 23: 72-80.
37. Goh NS, McDonald CE, MacGregor DP et al. Successful treatment of Langerhans cell histiocytosis with 2-chlorodeoxyadenosine. Respirology 2003; 8: 91-94.
38. Goto H, Inaba M, Kobayashi K et al. Seccessful treatment of multicentric reticulohistiocytosis with alendronate. Arthritism Rheumatism 2003; 48: 3538-3541.
39. Gotz G, Fichter J. Langerhans'-cell histiocytosis in 58 adults. Eur J Med Res 2004; 9: 510-514.
40. Grois N, Prayer D, Prosch H et al. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 2005; 128: 829-838.
41. Hale GA, Bowman LC, Woodard JP et al. Allogeneic bone marrow transplantation for children with histiocytic disorders: use of TBI and omission of etoposide in the conditioning regimen. Bone Marrow Transplant 2003; 31: 981-986.
42. Henter JI, Tondini C, Pritchard J. Histiocyte disorders. Crit Rev Oncol Hematol. 2004; 50: 157-174.
43. Henter JI. Succesful treatment of Langerhans cell histiocytosis with etanercept. N Engl J Med 2001; 345: 1577-1578.
44. Howarth DM, Mullan BP, Wiseman GA et al. Bone scintigraphy evaluated in diagnosis and staging Langerhans´s cell histiocytosis and related disorders. J Nuclear Med 1996; 37: 1456-1460.
45. Imashuku S, Ishida S, Koike K et al. Cerebellar ataxia in pediatric patients with Langerhans cell histiocytosis. J Pediatr Hematol Oncol 2004; 26: 735-739.
46. Jones B, Kung F, Chevalier L et al. Chemotherapy of retikuloendotheliosis. Comparison of methotrexate plus prednisone versus vincristine plus prednisone. Cancer 1974; 34: 1011-1017.
47. Jordan MB, McClain KL, Yan X et al. Anti-CD52 antibody, alemtuzumab, binds to Langerhans cells in Langerhans cell histiocytosis. Pediatr Blood Cancer 2005; 44: 251-254.
48. Kinugawa N, Imashuku S, Hirota Y et al. Hematopoietic stem cell transplantation (HSCT) for Langerhans cell histiocytosis (LCH) in Japan. Bone Marrow Transplant 1999; 24: 935-938.
49. Kolde G, Schulze P, Sterry W. Mixed response to thalidomide therapy in adults: two cases of multisystem Langerhans' cell histiocytosis. Acta Derm Venereol 2002; 82: 384-386.
50. Ladisch S, Gadner H, Arico M et al. LCH-1: A randomized trial of etoposide vs. vinblastine in disseminated Langerhans cell histiocytosis. Medical Pediatric Oncol 1994; 23: 107-110.
51. Lévy S, Capron D, Joly JP et al. Hepatic nodules as single organ involvement in an adult with Langerhans celle Granulomatosis. J Clin Gastroentrol 1998; 26: 69-73.
52. Malpas JS. Langerhans cell histiocytosis in adults. Hematol Oncol Clin North Amer 1998; 12: 259-268.
53. Mauro E, Fraulini C, Rigolin GM et al. A case of disseminated Langerhans' cell histiocytosis treated with thalidomide. Eur J Haematol 2005; 74: 172-174.
54. McClain KL. Drug therapy for the treatment of Langerhans cell histiocytosis. Expert Opin Pharmacother 2005; 6: 2435-2441.
55. Mendez JL, Nadrous HF, Vassallo R et al. Pneumothorax in pulmonary Langerhans cell histiocytosis. Chest 2004; 125: 1028-1032.
56. Meyer-Wentrup F, Foell J. Unrelated cord blood transplantation in an infant with severe multisystem Langerhans cell histiocytosis: clinical outcome, engraftment and culture of monocyte-derived dendritic cells. Bone Marrow Transplant 2004; 33: 875-876.
57. Minkov M, Grois N, Braier J et al. Immunosuppressive treatment for chemotherapy-resistant multisystem Langerhans cell histiocytosis. Med Pediatr Oncol 2003; 40: 253-256.
58. Minkov M, Grois N, Broadbent V et al. Cyclosporin A therapy for multisystem Langerhans cell histiocytosis. Med Pediatr Oncol 1999; 33: 482-485.
59. Minkov M, Grois N, Heitger A et al. Treatment of multisystem Langerhans cell histiocytosis. Results of the TAL HX83 and DAL-HX 90 studies. Klinische Pediatrie 2000; 212: 139-144.
60. Montella L, Insabato L, Palmieri G. Imatinib mesylate for cerebral Langerhans'-cell histiocytosis. N Engl J Med 2004; 351: 1034-1035.
61. Mottl H, Koutecký J, Ganevová M. Strategie léčby histiocytózy z Langerhansových buněk u dětí. Čes Slov Pediat 1994; 49: 81.
62. Mottl H, Mracek J, Kabelka Z et al. Histiocytóza z Langerhansových buněk u dětí. Čs Pediat 1992; 47: 530-533.
63. Munn S, Chu AC. Langerhans cell histiocytosis of the skin. Hematol Oncol Clin North Amer 1998; 12: 269-286.
64. Nagarajan R, Neglia J, Ramsay N et al. Successful treatment of refractory Langerhans cell histiocytosis with unrelated cord blood transplantation. J Pediatr Hematol Oncol 2001; 23: 629-632.
65. Nanduri VR, Lillywhite L, Chapman C et al. Cognitive outcome of long term survivors of multisystem Langerhans cell histiocytosis. J Clin Oncol 2003; 21: 2961-2967.
66. Nebelung W, Ropke M, Kluba U et al. Treatment concepts in osseous manifestations of Langerhans cell histiocytosis. Z Orthop Ihre Grenzgeb 1999; 137: 236-243.
67. Nicholson SH, Egeler M, Nesbit ME. The epidemiology of Langerhans cell histiocytosis. Hematol Oncol Clin North Amer 1998; 12: 379-348.
68. 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.
69. Pacovska V, Bortlova A, Homolka J et al. Granulomatóza z Langerhansových buněk. Trendy Med 2002; 4: 59-61.
70. Pardanani A, Phyliky R, Chin-Yang L et al. 2-chlordeoxyadenosine therapy for disseminated Langerhans cell histiocytosis. Mayo Clin Proc 2003; 78: 301-306.
71. Prosch H, Feldges A, Grois N et al. Demonstration of CD1a positive cells in the cerebrospinal fluid--A clue to diagnosis of isolated Langerhans cell histiocytosis of the hypothalamic-pituitary axis? Med Pediatr Oncol 2003; 41: 474-476.
72. Prosch H, Grois N, Prayer D et al. Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer 2004; 43: 594-599.
73. Putters TF, deVisscher JG, van Veen A et al. Intralesional infiltration of corticosteroids in the treatment of localised Langerhans´ cell histiocytosis of the mandibule. Int J Oral Maxillofac Surg 2005; 34: 571-575.
74. Reichle A, Vogt T, Kunz-Schughart L et al. Anti-inflammatory and angiostatic therapy in chemorefractory multisystem Langerhans' cell histiocytosis of adults. Br J Haematol 2005; 128: 730-732.
75. Rodriguez-Galindo C, Kelly P, Jeng M et al. Treatment of children with Langerhans cell histiocytosis with 2-chlorodeoxyadenosine. Am J Hematol 2002; 69: 179-184.
76. Rosen K, Jontell M, Mobacken H et al. Epidermal Langerhans-cells in chronic eczematous dermatitis of the palms treated with PUVA and UVB. Acta Derm Venereol 1989; 69: 200-205.
77. Rožánek P, Molnar V, Rešl M. Tři případy plicní granulomatózy z Langerhansových buněk. Lék Zpr Lék Fak Univ Karlovy Hr Králové 1998; 43: 127-132.
78. Sander CS, Kaatz M, Elsner P. Successful treatment of cutaneous langerhans cell histiocytosis with thalidomide. Dermatology 2004; 208: 149-152.
79. Sato Y, Ikeda Y, Ito E et al. Histiocytosis X: Successful treatment with recombinant interferon alpha-2a. Acta Paediatr Jpn 1990; 32: 151-154.
80. Saven A, Burian C. Cladribine activity in Langerhans cell histiocytosis. Blood 1999; 93: 4125-4130.
81. Saven A, Kenneth A, Foon A. 2-Chlorodeoxyadenosine induced complete remissions in Langerhans cell histiocytosis. Ann Intern Med 1994; 121: 430-433.
82. Šímová B, Mališ J, Neuwirt J. Klinické projevy histiocytózy z Langerhansových buněk. Zdrav Nov ČR Lék Listy 2003; 52: 18.
83. Skacel Z, Marel M, Vraštilova P et al. Histiocytóza z Langerhansových buněk. Přehled literatury a vlastní pozorování. Stud Pneumol Phtiseol 2000; 60: 150-156.
84. Smilek P, Krejčová B, Čada K et al. Histiocytóza z Langerhansových buněk, případ postižení spánkové kosti. Otorinolaryng Foniat 1994; 43: 263-265.
85. Steiner M, Matthes-Martin S, Attarbaschi A 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.
86. Steiner M, Prayer D, Asenbaum S et al. Modern imaging methods for the assessment of Langerhans' cell histiocytosis-associated neurodegenerative syndrome: case report. J Child Neurol 2005; 20: 253-257.
87. Stime KC, Saylors RL, Williams LL et al. 2-Chlordeoxyadenosine for the treatment of refractory or recurent Langerhans cell histiocytosis in pediatric patients. Medical Pediatric Oncol 1997; 29: 288-292.
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. Stoll M, Freund M, Schmid H et al. Allogeneic Bone Marrow transplantation for Langerhans cell histiocytosis. Cancer 1990; 66: 284-288.
90. Sulica R, Teirstein A, Padilla ML. Lung transplantation in interstitial lung disease. Curr Opin Pulm Med 2001; 7: 314-322.
91. Suminoe A, Matsuzaki A, Hattori H et al. Unrelated cord blood transplantation for an infant with chemotherapy-resistant progressive Langerhans cell histiocytosis. J Pediatr Hematol Oncol 2001; 23: 633-636.
92. Sundar KM, Gosselin MV, Chung HL et al. Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology, radiology and clinical evolution of the disease. Chest 2003; 123: 1673-1683.
93. Takemori H, Sakata Y, Suzuki H et al. A case of malignant histiocytosis succesfuly treated with combination of interferon alpha and etoposide therapy. Jpn J Clin Oncol 1990; 20: 431-435.
94. Thomas L, Ducros B, Secchi T et al. Successful treatment of adults Langerhans cell histiocytosis with thalidomide. Arch Dermatol 1993; 129: 1261-1264.
95. Tichy M jr, Tichy M, Krč I et al. Multicentrická retikulohistiocytóza. Ces Slov Derm 1999; 74: 168-171.
96. Toušovská K, Slavík Z. Histiocytóza z Langerhansových buněk v dětském věku. Lék Zprav Lék Fak Univ Karlovy Hr Králové 1997; 42: 127-132.
97. Tsambaos D, Georgiu S, Kapranos N et al. Langerhans cell histiocytosis: complete remission after oral Isotretinoid therapy. Acta Derm Venereol 1995; 75: 62-64.
98. Ugurel S, Pröhler G, Tilgen W et al. S100-β serum proein - a new marker in the diagnosis and monitoring of Langerhans cell histiocytosis. Brit J Dermatol 2000; 143: 201-202.
99. Večer J, Charvát J, Kubátová H et al. Sekundární hemofagocytující syndrom při systémovém onemocnění. Čas Lék Čes 2000; 139: 379-381.
100. Watts J, Files B. Langerhans cell histiocytosis: central nervous system involvement treated successfully with 2-chlorodeoxyadenosine. Pediatr Hematol Oncol 2001; 18: 199-204.
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