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Langerhans cell histiocytosis with histopathological features, single center experience

Year 2019, Volume: 7 Issue: Ek - IRUPEC 2019 Kongresi Tam Metin Bildirileri, 550 - 556, 10.12.2019

Abstract

Langerhans cell histiocytosis (LCH) is a rare histiocytic disease, occurring in 2-10 children per million and 1-2 adults per million, and may have a wide variety of clinical manifestations. Infiltration can develop in almost any organ (the most commonly reported organs are bone, skin, lymph nodes, lungs, thymus, liver, spleen, bone marrow and central nervous system). We aimed to evaluate the histopathological features of the lesions and review the literature in pediatric patients referred to our department for pathological examination and diagnosed as LCH.

Materials and Methods:
Retrospectively, childhood cases diagnosed with LCH in 2012-2019 were screened by hospital automation system. Age, gender, lesion localizations of the cases were recorded and histopathological features were reviewed.

Results:
5 male and 5 female total of 10 cases were detected. The youngest 3 were under the age of 1, the oldest was 16 years old. Localization; 6 of the cases were bone (2 femur, 3 skull bone, 1 scapula), 2 skin, 1 bone and lymph node, 1 lung and lymph node. Histopathology revealed histiocytic cells with grooved nuclei, eosinophilic cytoplasm with eosinophils, and neutrophils in some cases. Immunohistochemical CD1a staining was positive in all cases and positivities were present with S100 in applied 9 cases, CD68 in 4. Ki67 proliferation index was studied in 2 patients with bone localization, 15% and 20%.

Conclusion:
The term LCH is due to the morphological and immunophenotypic similarity of the infiltrating cells of this disease to Langerhans cells specialized as dendritic cells in the skin and mucous membranes. But these cells do not originate from the Langerhans cells of the skin, but from the myeloid progenitor cells of the bone marrow. Several studies have shown the BRAF-V600E mutation in LCH. The term LCH is currently recommended; histiocytosis-X, Letterer-Siwe disease, Hand-Schüller-Christian disease and diffuse reticuloendotheliosis were abandoned. The term eosinophilic granuloma may be used in the presence of a single lesion, especially in lytic bone lesions. As in our cases, it usually occurs with single or multiple osteolytic bone lesions and to a lesser extent with other organ involvement. It is characterized by infiltration of grooved nuclei histiocytes, accompanied by lymphocytes, neutrophils, macrophages and eosinophils, and areas of fibrosis and necrosis may develop. Immunohistochemical S100, CD1a, Langerin are positive, CD68 is variable. In the differential diagnosis, acute myelomonocytic leukemia, lymphoma, mastocytosis, osteomyelitis, sinus histiocytosis with massive lymphadenopathy should be considered.

References

  • References 1. Özkal S. Langerhans hücreli histiyositozis (langerhans hücreli granülomatozis, eozinofilik granülom, histiyositozis X). In: Dervişoğlu S, Bilgiç B, Doğanavşargil B, editors. Kemik ve eklem patolojisi multidisipliner yaklaşım. Ankara: Neyir Matbaacılık; 2018. p.383. 2. Uptodate.com [homepage on the Internet]. USA: Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis [updated 30 Jul 2019; cited Aug 2019]. Available from: uptodate.com 3. Unni KK, Inwards CY, Bridge JA, Kindblom L-G, Wold LE. Conditions that simulate primary neoplasms of bone. In: Silverberg SG, Sobin LH, editors. AFIP atlas of tumor pathology series 4, tumors of the bones and joints. Maryland: ARP Press; 2005. p. 321. 4. Weiss LM, Jaffe R, Facchetti F. Tumours derived from Langerhans cells. In: Swerdlow SH, Campo E, Harris NL et al, editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2017. p.470. 5. Berres ML, Lim KP, Peters T et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med 2014 Apr 7;211(4):669-83. 6. Jaffe R. Langerhans cell histiocytosis and langerhans cell sarcoma. In: Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber DA, editors. Hematopathology. Chine: Saunders; 2011. p.811. 7. Soyer T, Özyüksel G, Türer ÖB et al. Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report. European J Pediatr Surg Rep 2019 Jan;7(1):e8-e11. 8. Baumgartner I, von Hochstetter A, Baumert B, Luetolf U, Follath F. Langerhans'-cell histiocytosis in adults. Med Pediatr Oncol 1997 Jan;28(1):9-14. 9. Kim SS, Hong SA, Shin HC, Hwang JA, Jou SS, Choi SY. Adult Langerhans' cell histiocytosis with multisystem involvement: A case report. Medicine (Baltimore) 2018 Nov;97(48):e13366. 10. Goyal G, Ravindran A, Young JR et al. Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. Haematologica 2019 Apr 19. pii: haematol.2019.219626. 11. Milne P, Bigley V, Bacon CM et al. Hematopoietic origin of Langerhans cell histiocytosis and Erdheim-Chester disease in adults. Blood 2017 Jul 13;130(2):167-175.

Histopatolojik özellikleriyle langerhans hücreli histiyositoz, tek merkez deneyimi

Year 2019, Volume: 7 Issue: Ek - IRUPEC 2019 Kongresi Tam Metin Bildirileri, 550 - 556, 10.12.2019

Abstract

Amaç: LHH (Langerhans hücreli histiyositoz) nadir histiyositik bir hastalıktır, her yıl milyonda
2-10 çocukta ve milyonda 1-2 erişkinde karşılaşılır, oldukça çeşitli klinik tablolarla hemen her
organda infiltrasyon gelişebilir (en sık bildirilen organlar; kemik, deri, lenf nodları, akciğerler,
timus, karaciğer, dalak, kemik iliği ve santral sinir sistemidir). Bölümümüze patolojik inceleme
için gönderilen ve LHH tanısı alan pediatrik olgularda lezyonların histopatolojik özelliklerini
değerlendirmeyi ve literatür bilgilerini gözden geçirmeyi amaçladık.
Gereç ve Yöntem:
Retrospektif olarak, 2012-2019 yıllarında LHH tanısı alan çocukluk çağındaki olgular hastane
otomasyon sistemiyle taranarak tespit edildi. Olguların yaş, cinsiyet, lezyon lokalizasyonları,
histopatolojik özellikleri gözden geçirildi.
Bulgular:
5’i erkek, 5’i kız 10 olgu tespit edildi. En küçük 3’ü 1 yaşından küçüktü, en büyüğü 16
yaşındaydı. Lokalizasyon; olguların 6’sında kemik (2’sinde femur, 3’ünde kafa kemiği, 1’inde
skapula), 2’sinde deri, 1’inde kemik ve lenf nodu, 1’inde akciğer ve lenf noduydu.
Histopatolojilerinde tümünde çentikli nükleuslu, eozinofilik sitoplazmalı histiyositik hücreler
ve eozinofiller, bazı olgularda nötrofiller mevcuttu. Tüm olgularda immünohistokimyasal
CD1a pozitifti ve S100 uygulanan 9 olguda, CD68 uygulanan 4 olguda pozitiflik mevcuttu.
Ki67 proliferasyon indeksi kemik yerleşimli 2 olguda çalışılmıştı, %15 ve %20 oranlarındaydı.
Sonuç:
LHH terimi bu hastalıktaki infiltrasyonu oluşturan hücrelerin deri ve mukozalarda dendritik
hücreler olarak özelleşmiş Langerhans hücrelerine morfolojik ve immünfenotipik olarak
benzemeleri nedeniyledir. Fakat bu hücreler derinin Langerhans hücrelerinden değil kemik
iliğinin myeloid progenitör hücrelerinden köken alır. Çeşitli çalışmalarda LHH’da BRAFV600E mutasyonu gösterilmiştir. Günümüzde LHH terimi önerilmektedir; geçmişte kullanılan
histiyositozis-X, Letterer-Siwe hastalığı, Hand-Schüller-Christian hastalığı ve diffüz
retiküloendoteliozis terkedilmiştir. Tek lezyon varlığında, özellikle litik kemik lezyonunda
eozinofilik granülom terimi kullanılabilmektedir. Olgularımızdaki gibi, çoğunlukla tek veya
multipl osteolitik kemik lezyonlarıyla, daha az oranda diğer organ tutulumlarıyla ortaya çıkar.
Çentikli nükleuslu histiyositlerin infiltrasyonuyla karakterlidir, lenfositler, nötrofiller,
makrofajlar ve eozinofiller eşlik eder, fibrozis, nekroz gelişebilir. İmmünohistokimyasal S100,
CD1a, Langerin pozitiftir, CD68 değişkendir. Ayırıcı tanıda lokalizasyona göre akut
myelomonositik lösemi, lenfoma, mastositoz, osteomyelit, masif lenfadenopatili sinüs
histiyositoz düşünülmelidir.

References

  • References 1. Özkal S. Langerhans hücreli histiyositozis (langerhans hücreli granülomatozis, eozinofilik granülom, histiyositozis X). In: Dervişoğlu S, Bilgiç B, Doğanavşargil B, editors. Kemik ve eklem patolojisi multidisipliner yaklaşım. Ankara: Neyir Matbaacılık; 2018. p.383. 2. Uptodate.com [homepage on the Internet]. USA: Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis [updated 30 Jul 2019; cited Aug 2019]. Available from: uptodate.com 3. Unni KK, Inwards CY, Bridge JA, Kindblom L-G, Wold LE. Conditions that simulate primary neoplasms of bone. In: Silverberg SG, Sobin LH, editors. AFIP atlas of tumor pathology series 4, tumors of the bones and joints. Maryland: ARP Press; 2005. p. 321. 4. Weiss LM, Jaffe R, Facchetti F. Tumours derived from Langerhans cells. In: Swerdlow SH, Campo E, Harris NL et al, editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2017. p.470. 5. Berres ML, Lim KP, Peters T et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med 2014 Apr 7;211(4):669-83. 6. Jaffe R. Langerhans cell histiocytosis and langerhans cell sarcoma. In: Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber DA, editors. Hematopathology. Chine: Saunders; 2011. p.811. 7. Soyer T, Özyüksel G, Türer ÖB et al. Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report. European J Pediatr Surg Rep 2019 Jan;7(1):e8-e11. 8. Baumgartner I, von Hochstetter A, Baumert B, Luetolf U, Follath F. Langerhans'-cell histiocytosis in adults. Med Pediatr Oncol 1997 Jan;28(1):9-14. 9. Kim SS, Hong SA, Shin HC, Hwang JA, Jou SS, Choi SY. Adult Langerhans' cell histiocytosis with multisystem involvement: A case report. Medicine (Baltimore) 2018 Nov;97(48):e13366. 10. Goyal G, Ravindran A, Young JR et al. Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. Haematologica 2019 Apr 19. pii: haematol.2019.219626. 11. Milne P, Bigley V, Bacon CM et al. Hematopoietic origin of Langerhans cell histiocytosis and Erdheim-Chester disease in adults. Blood 2017 Jul 13;130(2):167-175.
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Details

Primary Language English
Subjects Health Care Administration
Journal Section Congress Proceedings
Authors

Fahriye Kılınç This is me

Publication Date December 10, 2019
Acceptance Date January 15, 2020
Published in Issue Year 2019 Volume: 7 Issue: Ek - IRUPEC 2019 Kongresi Tam Metin Bildirileri

Cite

Vancouver Kılınç F. Langerhans cell histiocytosis with histopathological features, single center experience. pediatr pract res. 2019;7(Ek):550-6.