Nadir Bir Pulmoner LHH Olgusu: Dört Aylık Bir İnfantta Tanıdan Taburculuğa Klinik İzlem

Öz

Langerhans hücreli histiyositoz (LHH), CD1a⁺/CD207⁺ hücrelerine farklılaşan miyeloid öncüllerin klonal çoğalması sonucu ortaya çıkarak çeşitli organ tutulumları ile bu organlarda işlev bozukluklarına yol açabilir. Bu çalışmada, dört aylık bir erkek infantta multisistemik LHH olgusu sunulmaktadır. Hasta, iki aylıkken papüloveziküler döküntüler nedeniyle birçok kez hastaneye başvurmuş ve çeşitli tanılarla tedavi edilmiş. Klinik yanıt alınamaması nedeniyle yapılan cilt biyopsisi ile hastaya LHH tanısı konuldu. Sistemik tutulum açısından yapılan değerlendirmelerde akciğerlerde çok sayıda kist ve nodüler lezyonlar tespit edildi. Hastaya tedavisi başlandı, takipte sitemegalovirus (CMV) enfeksiyonu ve pnömotoraks gibi komplikasyonlar gelişti. Bu olgu sunumu ile infant dönemde pulmoner tutulumlu LHH'nin nadir görülmesi açısından literatüre katkı sunulması amaçlanmaktadır.

Anahtar Kelimeler

• Langerhans Hücreli Histiyositoz
• Pulmoner Tutulum
• Pediatrik Olgu Sunumu

A Rare Case of Pulmonary LCH: Clinical Follow-up from Diagnosis to Discharge in a Four-Month-Old Infant

Öz

Langerhans cell histiocytosis (LCH) arises from the clonal expansion of myeloid precursors that differentiate into CD1a⁺/CD207⁺ cells, potentially leading to involvement of multiple organs and associated functional impairment. In this report, we present a case of multisystem LCH in a four-month-old male infant. The patient had been evaluated several times since two months of age due to papulovesicular skin eruptions and had received various treatments under different preliminary diagnoses. As no clinical improvement was achieved, a skin biopsy was performed and the diagnosis of LCH was established. Systemic evaluation revealed numerous cystic and nodular lesions in the lungs. Treatment was initiated; however, complications such as cytomegalovirus (CMV) infection and pneumothorax developed during follow-up. This case aims to contribute to the literature by

Anahtar Kelimeler

• Langerhans Cell Histiocytosis
• Pulmonary Involvement
• Pediatric Case Report.

Kaynakça

1. 1.Rodriguez-Galindo C, Allen CE, et al. Langerhanscell histiocytosis. Blood. 2020;135(16):1319-1331
2. 2.X Emile JF, Abla O, Fraitag S, et al. Revisedclassification of histiocytoses and neoplasms of themacrophage-dendritic cell lineages. Blood.2016;127(22):2672-2681
3. 3.Savaşan S. An enigmatic disease: childhoodLangerhans cell histiocytosis in 2005. Int J Dermatol.2006;45(3):182-188.
4. 4. Ye D, Yuan T, Jiang M. Langerhans cell histiocytosisin an infant. Pediatr Pulmonol. 2025;60(1): e27352.
5. 5.Minkov M. An update on the treatment ofpediatric-onset Langerhans cell histiocytosisthrough pharmacotherapy. Expert OpinPharmacother. 2018;19(3):233-242.
6. 6.Coşkun Ç, Kutluk T, Yalçın B. et al. Pulmonaryinvolvement in children with Langerhans cellhistiocytosis. Turkish Journal of Pediatrics.2024;66(3):323-331.
7. 7.Jezierska M, Stefanowicz J, Romanowicz G, et al.Langerhans cell histiocytosis in children - A diseasewith many faces. Recent advances in pathogenesis,diagnosticexaminations and treatment. PostepyDermatol Alergol. 2018;35(1):6-17
8. 8.Han D, Li F, Yahya WH, et al. MultisystemLangerhans Cell Histiocytosis in Younger InfantsFirst Presenting in Skin: A Case Series. Journal ofPersonalized Medicine. 2022; 12(7):1024.

9. 9.Piñana JL, Giménez E, Vázquez L, et al. Update OnCytomegalovırus Infectıon Management InAllogeneıc Hematopoıetıc Stem Cell TransplantRecıpıents: A Consensus Document of the SpanishGroup for Hematopoietic Transplantation and CellTherapy (GETH-TC). Mediterr J Hematol Infect Dis.2024;16(1):e2024065
10. 10.Downes KJ, Sharova A, Boge CLK. et al. CMVinfection and management among pediatric solidorgan transplant recipients. Pediatr Transplant.2022;26(3):e14220.