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Langerhans Cell Histiocytosis Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Adult LCH

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The most frequent pulmonary function abnormality finding in patients with pulmonary LCH is a reduced carbon monoxide diffusing capacity in 70% to 90% of cases.[12,17] A high-resolution computed tomography (CT) scan, which reveals a reticulonodular pattern classically with cysts and nodules, usually in the upper lobes and sparing the costophrenic angle, is characteristic of LCH. Despite the typical CT findings, most pulmonologists agree that a lung biopsy is needed to confirm the diagnosis.[18] The presence of cystic abnormalities on high-resolution CT scans appears to be a poor predictor of which patients will have progressive disease.[19] A study correlating lung CT findings and lung biopsy results in 27 pulmonary LCH patients has shed some light on pulmonary LCH.[20] Thin-walled and bizarre cysts had active LCs and eosinophils. Fifty-two percent of patients improved, most with smoking cessation, and some with steroid treatment within 14 months of diagnosis. Four patients (15%) were stable, and nine (33%) progressed over 22 months.

Liver

Liver involvement in adults has been reported in 27% of a series of adult LCH patients with multiorgan disease.[21] Hepatomegaly (48%) and liver enzyme abnormalities (61%) were present. CT and ultrasound imaging abnormalities are often found. The early histopathologic stage of liver LCH includes infiltration of CD1a+ cells and periductal fibrosis with inflammatory infiltrates with or without steatosis. The late stage is biliary tree sclerosis and treatment with ursodeoxycholic acid is suggested.

Multisystem disease

In a large series of patients from the Mayo Clinic, 31% had multisystem LCH compared with 69% registered on the Histiocyte Society adult registry; this likely reflects referral bias.[5,22] In the adult multisystem patients, the organs involved include the following:

  • Skin (50%).
  • Mucocutaneous (40%).
  • Diabetes insipidus (29.6%).
  • Hepatosplenomegaly (16%).
  • Hypothyroidism (6.6%).
  • Lymphadenopathy (6%).

References:

  1. Baumgartner I, von Hochstetter A, Baumert B, et al.: Langerhans'-cell histiocytosis in adults. Med Pediatr Oncol 28 (1): 9-14, 1997.
  2. Götz G, Fichter J: Langerhans'-cell histiocytosis in 58 adults. Eur J Med Res 9 (11): 510-4, 2004.
  3. Slater JM, Swarm OJ: Eosinophilic granuloma of bone. Med Pediatr Oncol 8 (2): 151-64, 1980.
  4. Kaltsas GA, Powles TB, Evanson J, et al.: Hypothalamo-pituitary abnormalities in adult patients with langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment. J Clin Endocrinol Metab 85 (4): 1370-6, 2000.
  5. Aricò M, Girschikofsky M, Généreau T, et al.: Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer 39 (16): 2341-8, 2003.
  6. Vassallo R, Ryu JH, Schroeder DR, et al.: Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med 346 (7): 484-90, 2002.
  7. Schönfeld N, Frank W, Wenig S, et al.: Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 60 (1): 38-44, 1993.
  8. Travis WD, Borok Z, Roum JH, et al.: Pulmonary Langerhans cell granulomatosis (histiocytosis X). A clinicopathologic study of 48 cases. Am J Surg Pathol 17 (10): 971-86, 1993.
  9. Tazi A, Moreau J, Bergeron A, et al.: Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. J Immunol 163 (6): 3511-5, 1999.
  10. Yousem SA, Colby TV, Chen YY, et al.: Pulmonary Langerhans' cell histiocytosis: molecular analysis of clonality. Am J Surg Pathol 25 (5): 630-6, 2001.
  11. Vassallo R, Ryu JH, Colby TV, et al.: Pulmonary Langerhans'-cell histiocytosis. N Engl J Med 342 (26): 1969-78, 2000.
  12. Delobbe A, Durieu J, Duhamel A, et al.: Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Groupe d'Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord. Eur Respir J 9 (10): 2002-6, 1996.
  13. Chaulagain CP: Pulmonary langerhans' cell histiocytosis. Am J Med 122 (11): e5-6, 2009.
  14. Lin MW, Chang YL, Lee YC, et al.: Pulmonary Langerhans cell histiocytosis. Lung 187 (4): 261-2, 2009.
  15. Tazi A, Hiltermann J, Vassallo R: Adult lung histiocytosis. In: Weitzman S, Egeler R M, eds.: Histiocytic Disorders of Children and Adults. Cambridge, United Kingdom: Cambridge University Press, 2005, pp 187-207.
  16. Dauriat G, Mal H, Thabut G, et al.: Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis. Transplantation 81 (5): 746-50, 2006.
  17. Crausman RS, Jennings CA, Tuder RM, et al.: Pulmonary histiocytosis X: pulmonary function and exercise pathophysiology. Am J Respir Crit Care Med 153 (1): 426-35, 1996.
  18. Diette GB, Scatarige JC, Haponik EF, et al.: Do high-resolution CT findings of usual interstitial pneumonitis obviate lung biopsy? Views of pulmonologists. Respiration 72 (2): 134-41, 2005 Mar-Apr.
  19. Soler P, Bergeron A, Kambouchner M, et al.: Is high-resolution computed tomography a reliable tool to predict the histopathological activity of pulmonary Langerhans cell histiocytosis? Am J Respir Crit Care Med 162 (1): 264-70, 2000.
  20. Kim HJ, Lee KS, Johkoh T, et al.: Pulmonary Langerhans cell histiocytosis in adults: high-resolution CT-pathology comparisons and evolutional changes at CT. Eur Radiol 21 (7): 1406-15, 2011.
  21. Abdallah M, Généreau T, Donadieu J, et al.: Langerhans' cell histiocytosis of the liver in adults. Clin Res Hepatol Gastroenterol 35 (6-7): 475-81, 2011.
  22. Howarth DM, Gilchrist GS, Mullan BP, et al.: Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer 85 (10): 2278-90, 1999.
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Last Updated: February 25, 2014
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