Langerhans Hücreli Histiyositozun Patolojisi, Sınıflandırılması, Klinik Belirtileri ve Prognozu: Tek Merkez Deneyimi
Abstract
Amaç: Çalışmanın amacı, nadir görülen bu hastalığın; klinik özellikler, histopatolojik, radyolojik analizler ve tedavi detayları hakkında farkındalığı arttırmaktır.
Materyal method: 2006 Ocak-2020 Ekim tarihleri arasında anabilim dalımızda tanı konan 55 Langerhans hücre histiyositozu hastası çalışmaya dahil edildi. Hastalar yaş, cinsiyet, lokalizasyon, risk grupları, tıbbi tedavi, nüks ve hastalığın sonuçları açısından değerlendirildi.
Sonuçlar: 55 hastanın 23’ü çocuk, 32'si yetişkindi. Hastaların yaşları 7 ay ile 72 yıl arasında değişmektedir. Olguların 37’si erkek, 18'i kadındı. Her iki gruptada en sık şikâyet ağrı ve şişlikti. Hasta şikâyeti ile hastaneye başvuru süresi çocuklarda 7 gün ile 12 ay arasında değişirken, erişkinlerde 10 gün ile 23 yıl arasında değişmektedir. Olguların 43'ünde tek organ tutulumu, 12'sinde multiorgan tutulumu vardı. Yetişkinlerde ve çocuklarda en sık etkilenen organ kemikti. Takipli hastalar tedavi açısından incelendiğinde: 9 olgu radyoterapi, 8 olgu kemoterapi + steroid, 7 olgu kemoterapi, 2 olgu kemoterapi + radyoterapi + steroid, 1 olgu sadece steroid, 2 olgu kemoterapi + radyoterapi ve onbir olgu ise cerrahi sonrası ek tedavi gerekmeksizin takip edildi. Biyopsiden sonra medyan takip süresi çocuklarda 45.9 ay ve erişkinlerde 41.2 ay idi.
Sonuç olarak tanı için yüksek derecede şüphe gerektiren hastalıkta, kesin tanı lezyonların ve biyopsilerin histolojik incelemesine dayanmaktadır.
Supporting Institution
Başkent Üniversitesi Tıp Fakültesi
Project Number
KA21/444
References
- Referans1-Lee JW, Shin HY, Kang HJ, Kim H, Park JD, Park KD, et al. Clinical Characteristics and Treatment Outcome of Langerhans Cell Histiocytosis: 22 Years’ Experience of 154 Patients at a Single Center. Pediatric Hematology and Oncology. 2014;31:293-302.
- Referans2-Kamer SA, Kıraklı EK, Çetingül N, Kantar M, Saydam G, Anacak Y. Langerhans Cell Histiocytosis: Excellent Local Control with Low Dose Radiotherapy. International Journal of Hematology and Oncology. 2019;29:7-13.
- Referans3-Brito MD, Martins A, Andrade J, Guimaraes J, Mariz J. Adulthood Langerhans Cell Histiocytosis: Experience of Two Portuguese Hospitals. Acta Med Port. 2014;27:726-30.
- Referans4-Tokgöz H, Çalışkan U. Langerhans Cell Histiocytosis in children: A single Center Experience from Turkey. International Journal of Hematology and Oncology. 2016;26: 83-88.
- Referans5-Kapukaya A, Işık R, Alemdar C, Yıldırım A. Langerhans-hücreli histiyositoz. TOTBİD Dergisi. 2013;12:547-56.
- Referans6-İnce D, Demirağ B, Özek G, Erbay A, Ortaç R, Oymak Y, et al. Pediatric langerhans cell histiocytosis: single-center experience over a 17-year period. The Turkish Journal of Pediatrics. 2016;58:349-55.
- Referans7-Özer E, Sevinc A, İnce D, Yüzügüldü R, Olgun N. BRAF V600E Mutation: A Significant Biomarker for Prediction of Disease Relapse in Pediatric Langerhans Cell Histiocytosis. Pediatr Dev Pathol. 2019;22:449-55.
- Referans8-Kambouchner M, Emile JF, Copin MC, Lhermine AC, Sabourin JC, Valle VD, et al. Childhood pulmonary Langerhans cell histiocytosis: a comprehensive clinical – histopathological and BRAF mutation study from the French national cohort. Hum Pathol. 2019;89:51-61.
- Referans9-Singh T, Satheesh CT, Appaji L, Kumari BS, Mamatha HS, Giri GV, et al. Langerhan’s cell histiocytosis: A single institutional experience. Indian Journal of Medical and Pediatric Oncology. 2010;31;51-53.
- Referans10-Aydoğdu K, Günay E, Fındık G, Günay S, Ağaçkıran Y, Kaya S, et al. Pulmonary Langerhans cell histiocytosis; characteristics of 11 cases. Tuberk Toraks. 2013;61:333-41.
- Referans11-İnci R, Sayar H, İnci MF, Öztürk P. Erişkin Başlangıçlı Langerhans Hücreli Histiyositoz. Türk J Dermatol. 2014;4:236-39.
- Referans12-Çelik B, Furtun K, Bilgin S. Pulmoner Langerhans Hücreli Histiyositoz. Turk Toraks Der. 2010;11:84-86.
- Referans13-Gülhan PY, Ekici A, Bulcun E, Ekici MS. Pulmoner langerhans hücreli histiyositosiz x: Dört olgunun analizi. Respir case rep. 2013;2:106-11.
- Referans14-Poompuen S, Chaiyarit J, Techasatian L. Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study. European Journal of Pediatrics. 2019;178:771-76.
- Referans15-Chellapandian D, Hines MR, Zhang R, Jeng M, Bos, C, Maria Lopez VS, et al. A Multicenter Study of Patients with Multisystem Langerhans Cell Histiocytosis Who Develop Secondary Hemophagocytic Lymphohistiocytosis. Cancer. 2019;125:963-71.
- Referans16-Düzenli Ö, Emlik GD, Kireşi D. Multisistemik Langerhans Hücreli Histiyositozis Hastalığında BT ve MR Bulguları. Selçuk Üniv Tıp Derg. 2011;27:118-20.
- Referans17-Lomora P, Simonetti I, Vinci G, Fichera V, Tarotto P, Prevedoni Gorone MS. Secondary aneurysmal bone cyst in Langerhans cell histiocytosis: Case report, literature review. European Journal of Radiology 0pen. 2019;6:97-100.
- Referans18-Durdu M, Koçer NE. Persistent Napkin Dermatitis: Langerhans Cell Histiocytosis. Clin Oncol. 2018;3:1493.
- Referans19-Asilsoy S, Yazıcı N, Demir, Ş, Erbay A, Koçer E, Sarıalioğlu F. A different cause for respiratory disorder in children: cases with pulmonary Langerhans cell histiocytosis. Clin Respir Jl. 2017;11:193-99.
- Referans20-Türk M, Türktaş H, Akyürek N. A case of Langerhans Cell Histiocytosis with Atypical Radiological Presentation. Turk Toraks Derg. 2015;16:154-56.
- Referans21- Al Hamad MA, Albisher HM, Al Saeed WR, Almumtin AT, Allabbad FM, Shawarby MA. BRAF gene mutations in synchronous papillary thyroid carcinoma and Langerhans cell histiocytosis co-existing in the thyroid gland: a case report and literature review. BMC Cancer. 2019;19:170-76.
- Referans22- Kim SS, Hong SA, Shin HC, Hwang JA, Jou SS, Choi SY. Adult Langerhans’ cell histiocytosis with multisystem involvement. Medicine. 2018;48:13366.
- Referans23-Matsumoto N, Toriumi N, Sarashina T, Hatakeyama N, Azuma H. Langerhans cell histiocytosis isolated to the thymus in a 7‐month‐old infant. Pediatrics International. 2019;61:205-06.
- Referans24-Lee HB, George S, Kutok JL. Langerhans cell histiocytosis involving the thymus. A case report and review of the literature. Arch Pathol lab med. 2003;127:294-97.
Pathology, Classification, Clinical Manifestations and Prognosis of Langerhan’s Cell Histiocytosis: A Single Center Experience
Abstract
Objective: The aim of the study is to raise awareness about clinical features,
histopathological and radiological analyzes and treatment details of this rare
disease.
Methods: A total of 55 Langerhans cell histiocytosis patients, diagnosed
between the years 2006 and October 2020 in our department were included in the
study. The patients were evaluated in terms of age, gender, tumor localization,
risk groups, treatment modalities, recurrence, and disease outcome.
Results: Twenty-three out of 55 patients were children and 32 were adults. The
ages of the patients were between 7 months and 72 years. Thirty-seven of the
cases were male and 18 were female. The most common clinical complaint in
both groups was pain and swelling. The duration between the onset of the patient
complaints and admission to the hospital varies between 7 days-12 months in
children, and 10 days-23 years in adults. Forty-three of the cases had single-organ
involvement and 12 had multiorgan involvement. The most frequently affected
organ in both groups was bone. Forty of the 55 patients had follow-up data and
the treatment modalities are as follows: Nine patients radiotherapy, 8 patients
chemotherapy+steroid, 7 patients chemotherapy, 2 patients
chemotherapy+radiotherapy+steroid, 1 patient steroid, and 2 patients
chemotherapy+radiotherapy. Eleven patients were followed up without
additional treatment after surgery. Median follow-up from the time of biopsy
was 45.9 months in children and 41.9 months in adults.
Conclusions: As a result, diagnosis requires a high degree of suspicion and final
diagnosis is based on the histological examination of the lesions and biopsies
Project Number
KA21/444
References
- Referans1-Lee JW, Shin HY, Kang HJ, Kim H, Park JD, Park KD, et al. Clinical Characteristics and Treatment Outcome of Langerhans Cell Histiocytosis: 22 Years’ Experience of 154 Patients at a Single Center. Pediatric Hematology and Oncology. 2014;31:293-302.
- Referans2-Kamer SA, Kıraklı EK, Çetingül N, Kantar M, Saydam G, Anacak Y. Langerhans Cell Histiocytosis: Excellent Local Control with Low Dose Radiotherapy. International Journal of Hematology and Oncology. 2019;29:7-13.
- Referans3-Brito MD, Martins A, Andrade J, Guimaraes J, Mariz J. Adulthood Langerhans Cell Histiocytosis: Experience of Two Portuguese Hospitals. Acta Med Port. 2014;27:726-30.
- Referans4-Tokgöz H, Çalışkan U. Langerhans Cell Histiocytosis in children: A single Center Experience from Turkey. International Journal of Hematology and Oncology. 2016;26: 83-88.
- Referans5-Kapukaya A, Işık R, Alemdar C, Yıldırım A. Langerhans-hücreli histiyositoz. TOTBİD Dergisi. 2013;12:547-56.
- Referans6-İnce D, Demirağ B, Özek G, Erbay A, Ortaç R, Oymak Y, et al. Pediatric langerhans cell histiocytosis: single-center experience over a 17-year period. The Turkish Journal of Pediatrics. 2016;58:349-55.
- Referans7-Özer E, Sevinc A, İnce D, Yüzügüldü R, Olgun N. BRAF V600E Mutation: A Significant Biomarker for Prediction of Disease Relapse in Pediatric Langerhans Cell Histiocytosis. Pediatr Dev Pathol. 2019;22:449-55.
- Referans8-Kambouchner M, Emile JF, Copin MC, Lhermine AC, Sabourin JC, Valle VD, et al. Childhood pulmonary Langerhans cell histiocytosis: a comprehensive clinical – histopathological and BRAF mutation study from the French national cohort. Hum Pathol. 2019;89:51-61.
- Referans9-Singh T, Satheesh CT, Appaji L, Kumari BS, Mamatha HS, Giri GV, et al. Langerhan’s cell histiocytosis: A single institutional experience. Indian Journal of Medical and Pediatric Oncology. 2010;31;51-53.
- Referans10-Aydoğdu K, Günay E, Fındık G, Günay S, Ağaçkıran Y, Kaya S, et al. Pulmonary Langerhans cell histiocytosis; characteristics of 11 cases. Tuberk Toraks. 2013;61:333-41.
- Referans11-İnci R, Sayar H, İnci MF, Öztürk P. Erişkin Başlangıçlı Langerhans Hücreli Histiyositoz. Türk J Dermatol. 2014;4:236-39.
- Referans12-Çelik B, Furtun K, Bilgin S. Pulmoner Langerhans Hücreli Histiyositoz. Turk Toraks Der. 2010;11:84-86.
- Referans13-Gülhan PY, Ekici A, Bulcun E, Ekici MS. Pulmoner langerhans hücreli histiyositosiz x: Dört olgunun analizi. Respir case rep. 2013;2:106-11.
- Referans14-Poompuen S, Chaiyarit J, Techasatian L. Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study. European Journal of Pediatrics. 2019;178:771-76.
- Referans15-Chellapandian D, Hines MR, Zhang R, Jeng M, Bos, C, Maria Lopez VS, et al. A Multicenter Study of Patients with Multisystem Langerhans Cell Histiocytosis Who Develop Secondary Hemophagocytic Lymphohistiocytosis. Cancer. 2019;125:963-71.
- Referans16-Düzenli Ö, Emlik GD, Kireşi D. Multisistemik Langerhans Hücreli Histiyositozis Hastalığında BT ve MR Bulguları. Selçuk Üniv Tıp Derg. 2011;27:118-20.
- Referans17-Lomora P, Simonetti I, Vinci G, Fichera V, Tarotto P, Prevedoni Gorone MS. Secondary aneurysmal bone cyst in Langerhans cell histiocytosis: Case report, literature review. European Journal of Radiology 0pen. 2019;6:97-100.
- Referans18-Durdu M, Koçer NE. Persistent Napkin Dermatitis: Langerhans Cell Histiocytosis. Clin Oncol. 2018;3:1493.
- Referans19-Asilsoy S, Yazıcı N, Demir, Ş, Erbay A, Koçer E, Sarıalioğlu F. A different cause for respiratory disorder in children: cases with pulmonary Langerhans cell histiocytosis. Clin Respir Jl. 2017;11:193-99.
- Referans20-Türk M, Türktaş H, Akyürek N. A case of Langerhans Cell Histiocytosis with Atypical Radiological Presentation. Turk Toraks Derg. 2015;16:154-56.
- Referans21- Al Hamad MA, Albisher HM, Al Saeed WR, Almumtin AT, Allabbad FM, Shawarby MA. BRAF gene mutations in synchronous papillary thyroid carcinoma and Langerhans cell histiocytosis co-existing in the thyroid gland: a case report and literature review. BMC Cancer. 2019;19:170-76.
- Referans22- Kim SS, Hong SA, Shin HC, Hwang JA, Jou SS, Choi SY. Adult Langerhans’ cell histiocytosis with multisystem involvement. Medicine. 2018;48:13366.
- Referans23-Matsumoto N, Toriumi N, Sarashina T, Hatakeyama N, Azuma H. Langerhans cell histiocytosis isolated to the thymus in a 7‐month‐old infant. Pediatrics International. 2019;61:205-06.
- Referans24-Lee HB, George S, Kutok JL. Langerhans cell histiocytosis involving the thymus. A case report and review of the literature. Arch Pathol lab med. 2003;127:294-97.
Details
| Primary Language | English |
|---|---|
| Subjects | Health Care Administration |
| Journal Section | Articles |
| Authors | |
| Project Number | KA21/444 |
| Publication Date | October 20, 2022 |
| Acceptance Date | September 5, 2022 |
| Published in Issue | Year 2022 Volume: 14 Issue: 3 |
