General Information
        Incidence
        Presentation of Adult Langerhans Cell Histiocytosis by Organ, Site or System

General Information

Incidence

It is estimated that one to two adult cases of Langerhans cell histiocytosis (LCH) occur per million population.[1] The true incidence of this disease is impossible to know because large published studies usually are from referral centers and the disorder often is under-diagnosed. A survey from Germany reported that 66% of the LCH patients were women with an average age of 43.5 years for all patients.[2]

Adult LCH patients may have symptoms and signs for many months before a definitive diagnosis and treatment. LCH in adults is often similar to that in children, except that adult pulmonary LCH is closely associated with smoking. Adult pulmonary LCH has some unique biologic charcteristics.

Presenting symptoms from published studies are (in order of decreasing frequency) dyspnea or tachypnea, polydipsia and polyuria, bone pain, lymphadenopathy, weight loss, fever, gingival hypertrophy, ataxia, and memory problems. Among the signs of LCH are skin rash, scalp nodules, soft tissue swelling near bone lesions, lymphadenopathy, gingival hypertrophy, and hepatosplenomegaly. Patients who present with isolated diabetes insipidus (DI) should be carefully observed for onset of other symptoms or signs characteristic of LCH. At least 80% of patients with DI had involvement of other organ systems, i.e., bone (68%), skin (57%), lung (39%), and lymph nodes (18%).[3]

Many patients have a papular rash with brown, red, or crusted areas ranging from the size of a pinhead to a dime. In the scalp, the rash is similar to that of seborrhea. Skin in the inguinal region, genitalia, or around the anus may have open ulcers that do not heal after antibacterial or antifungal therapy. In the mouth, swollen gums or ulcers along the cheeks, roof of the mouth or tongue may be signs of LCH.

The relative frequency of bone involvement in adults differs from that in children, e.g., mandible (30% vs. 7%) and skull (21% vs. 40%).[1-4] The frequency in adults of vertebrae (13%), pelvis (13%), extremities (17%), and rib (6%) lesions are similar to those found in children.[1]

Pulmonary LCH is slightly more prevalent in smokers than in nonsmokers and the male/female ratio may be near unity depending on the incidence of smoking in the population studied.[5,6] Patients with pulmonary LCH usually present with cough, dyspnea, or chest pain, although nearly 20% of adults with lung involvement have no symptoms.[7,8] The presence of chest pain may indicate the presence of a spontaneous pneumothorax. The Langerhans cells (LCs) in adult lung lesions were shown to be mature dendritic cells expressing high levels of the accessory molecules CD80 and CD86, unlike LCs found in other lung disorders.[8] In addition, pulmonary LCH in adults appears to be primarily a reactive process, rather than a clonal proliferation as seen in childhood LCH.[9]

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.[10,11] A high-resolution computed tomography (CT) scan, which reveals cysts and nodules, usually in the upper lobes, are characteristic of LCH (see the Pulmonary Langerhans cell histiocytosis section on Computed tomography). Despite the typical CT findings, most pulmonologists agree that a lung biopsy is needed to confirm the diagnosis.[12] The presence of cystic abnormalities on high-resolution CT scans appears to be a poor predictor of which patients will have progressive disease.[13]

Adults with LCH can have multisystem disease including bone (18%), skin (13%), and DI (5%) in those with pulmonary disease.

References

1. Baumgartner I, von Hochstetter A, Baumert B, et al.: Langerhans'-cell histiocytosis in adults. Med Pediatr Oncol 28 (1): 9-14, 1997.  [PUBMED Abstract]
   
   
2. Götz G, Fichter J: Langerhans'-cell histiocytosis in 58 adults. Eur J Med Res 9 (11): 510-4, 2004.  [PUBMED Abstract]
   
   
3. 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.  [PUBMED Abstract]
   
   
4. Slater JM, Swarm OJ: Eosinophilic granuloma of bone. Med Pediatr Oncol 8 (2): 151-64, 1980.  [PUBMED Abstract]
   
   
5. 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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
7. 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.  [PUBMED Abstract]
   
   
8. 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.  [PUBMED Abstract]
   
   
9. 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.  [PUBMED Abstract]
   
   
10. 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.  [PUBMED Abstract]
    
    
11. 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.  [PUBMED Abstract]
    
    
12. 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.  [PUBMED Abstract]
    
    
13. 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.  [PUBMED Abstract]
    
    

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