Pulmonary Langerhans' Cell Histiocytosis: Report of Six Cases

Öz

Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare pulmonary interstitial disease. In this report between January 2004 and December 2009 treated by the Dicle University Faculty of Medicine PLCH patients records were retrospectively studied. We found six PLCH patients. Two of six were female and four male. Mean age was 28,2± 7,3. There were only lung invoment in three, lungs and the pituitary involvement in two, lung,and skin involvement in one. Diagnosis of PLCH was established by video-assisted thoracoscopic surgery in three cases, by open lung biopsy in two and in a case by skin biopsy. There were smoking history in five cases. Dyspnea and cough were the most frequent symptoms. Most obvious lung function test paterns were obstruction and DLCO diminished. Most seen radiological abnormalities were cystic lesions on chest X-ray and nodulary-cystic lesions on HRCT. Two of them were upon cessation of smoking. Prednisolone was given three patients. Complete remission was observed and steroid therapy was stopped in six months. Desmopressin was started for two patients with diabetes insipidus and complete clinical improvement was observed after one month . PLCH should be considered when smoking history with young patient and multiple cystic lesions radiographycally present. It should be awaiken in the view of other organ involvement.

Anahtar Kelimeler

• Langerhans cell histiocytosis, Cigarette, High resolution computed tomography

Pulmoner Langerhans Hücreli Histiyositozis: Altı Olgunun İncelenmesi

Öz

Pulmoner Langerhans Hücreli Histiyositozis (PLHH), nadir görülen bir interstisyel akciğer hastalığıdır. Bu yazıda Ocak 2004-Aralık 2009 tarihleri arasında Dicle Üniversitesi Tıp Fakültesinde tedavi edilen altı PLHH hastasının özellikleri irdelendi. Hastaların ikisi kadın, dördü erkekti ve ortalama yaş 28,2± 7,3 idi. Olguların üçünde sadece akciğer, ikisinde akciğer ve hipofiz, birinde ise akciğer ve cilt tutulumu vardı. PLHH tanısı, hastaların üçünde video-yardımlı torakoskopik biyopsi ile, ikisinde açık akciğer biyopsi ile ve birinde cilt biyopsisi ile kondu. Olguların beşinde sigara öyküsü olup, en belirgin şikayetler nefes darlığı ve kuru öksürüktü. Solunum fonksiyon testinde en belirgin patern obstrüksiyon ve karbon monoksit difüzyon kapasitesinde azalmaydı. Akciğer grafilerinde en sık yaygın kistik lezyonlar, yüksek rezolüsyonlu bilgisayarlı tomografide yaygın nodüler-kistik lezyonlar gözlendi. Olgulardan ikisine sigara bıraktırıldı. Üçüne metil prednisolon başlandı ve altı ayda tam remisyon yanıtı saptanarak steroid tedavi-leri kesildi. Diabetes insipitus saptanan iki hastaya desmopressin başlandı, bir ay sonra tam klinik düzelme gözlendi. Özellikle sigara öyküsü olan genç hastalarda, yaygın kistik lezyonların gözlendiği radyolojik bulguların varlığında PLHH düşünülmeli, diğer organ tutulumları açısından dikkatli olunmalıdır.

Anahtar Kelimeler

• Pulmoner langerhans hücreli histiyositozis, Sigara, Yüksek rezolüsyonlu bilgisayarlı tomografi

Kaynakça

1. Vassallo R, Ryu JH, Colby TV, et al. Pulmonary Langerhanscell histiocytosis. N Engl J Med 2000; 342: 1969-78.
2. Suri HS, Yi ES, Nowakowski GS, et al. Pulmonary Langerhans Cell Histiocytosis Orphanet J Rare Dis 2012; 7: 16.
3. Thomeer M, Demedts M, Vandeurzen K. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin Belg 2001; 56: 163-72.
4. Watanabe R, Tatsumi K, Hashimoto S, et al. Clinicoepidemiological features of pulmonary histiocytosis X. Intern Med 2001; 40: 998-1003.
5. Rao RN, Goodman LR, Tomashefski JF Jr. Smoking-related interstitial lung disease. Ann Diagn Pathol 2008; 12: 445-57.
6. Crausman RS, Jennings CA, Tuder RM, et al. Pulmonaryhistiocytosis X: pulmonary function and exercise pathophysiology. Am J Respir Crit Care Med 1996; 153: 426-35.
7. Aguayo SM. Kane MA, King TE Jr et al. Increased levels of bombesin-like peptides in the lower respiratory tract of asymptomatic cigarette smokers. J Clin Invest 1989; 84: 1105-13.
8. Vassallo R, Ryu JH, Schroeder DR, et al. Clinical outcomes of pulmonary Langerhans’-cell histiocytosis in adults. N Engl J Med 2002; 346: 484-90.

9. Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of lung. Medicine 1981; 60: 385-96.
10. Sundar KM, Gosselin MV, Chung HL, et al. Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology, radiology, and clinical evolution of disease. Chest 2003; 123: 1673-81.
11. Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans’cell histiocytosis. Thorax 2000; 55: 405-16.
12. Abi Khalil S, Gourdier AL, Aoun N,et al. Cystic and cavitary lesions of the lung: imaging characteristics and differential diagnosis. J Radiol 2010; 91: 465-73.
13. 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 2011; 21: 1406
14. Mendez JL, Nadrous HF, Vassallo R, et al. Pneumothorax in pulmonary Langerhans cell histiocytosis. Chest 2004; 125: 1028
15. Demedts M, Wells AU, Anto JM, et al. Interstitial lung diseases: an epidemiological overview. Eur Respir J Suppl 2001; 32: 2-16.
16. Diette GB, Scatarige JC, Haponik EF,et al. Do high-resolution CT findings of usual interstitial pneumonitis obviate lung biopsy? Views of pulmonologists. Respiration 2005; 72: 134-41.
17. Torre O, Harari S. The diagnosis of cystic lung diseases: a role for bronchoalveolar lavage and transbronchial biopsy? Respir Med 2010; 104: 81-5.
18. Auerswald U, Barth J, Magnussen H. Value of CD-1-positive cells in bronchoalveolar lavage fluid for thediagnosis of pulmonary histiocytosis X. Lung 1991; 169: 305-9.
19. Liang GC, Granston AS. Complete remission of multicentric reticulohistiocytosis with combi- nation therapy of steroid, cyclophosphamide, and low-dose pulse methotrexate. Case report, review of the literature, and proposal for treatment. Arthritis Rheum 1996; 39: 171-4.
20. Akcay S, Eyuboğlu F, Arıcan A ve ark. Pulse steroid tedavisinin Langerhans hücreli histiyositozis olgusundaki etkinliği. Solunum Hastalıkları 2000; 11: 419-25.
21. Kömürcüoğlu B, Yalnız E, Büyükşirin M ve ark. Spontan regresyon izlenen pulmoner eozinofilik granuloma. Solunum 2004; 6: 84-8. Gönderilme Tarihi: 10.08.2012