Recurrent Multifocal Langerhans Cell Histiocytosis With Orbital Manifestation

Praba, Venansya Maulina; Faiqoh, Maimanah Zumaro Ummi; Kamal, Ronik Harsono; Fatmariyanti, Susy

doi:10.61186/ijbc.17.1.1

Volume 17, Issue 1 (March-2025 2025) Iranian Journal of Blood and Cancer 2025, 17(1): 1-12 | Back to browse issues page

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Praba V M, Faiqoh M Z U, Kamal R H, Fatmariyanti S. Recurrent Multifocal Langerhans Cell Histiocytosis With Orbital Manifestation. Iranian Journal of Blood and Cancer 2025; 17 (1) :1-12
URL: http://ijbc.ir/article-1-1684-en.html

Recurrent Multifocal Langerhans Cell Histiocytosis With Orbital Manifestation

Venansya Maulina Praba ^*¹

, Maimanah Zumaro Ummi Faiqoh¹

, Ronik Harsono Kamal²

, Susy Fatmariyanti³

1- Faculty of Medicine Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, Indonesia.
2- Faculty of Medicine Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, Indonesia. & Department of ophthalmology, Faculty of Medicine Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, Indonesia.
3- Faculty of Medicine Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, Indonesia. & Reconstruction, Oncology, and Oculoplasty Division, Departmant of Ophthalmology, Faculty of Medicine Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, Indonesia.

Abstract: (801 Views)

Background: Langerhans Cell Histiocytosis (LCH) is a rare disease and is more common in children than adults. The incidence is estimated to be approximately 5–10 cases per million children annually. LCH has a wide range of clinical manifestations with rare orbital involvement.
Case Presentation: Here, we report a six year-old-girl with recurrent multifocal LCH a year after chemotherapy accompanied by orbital manifestation, present as proptosis of left eye, lagophthalmos, and left superior palpebral abscess with fistula. She had a history of occipital brain LCH with bone involvement, which was surgically removed four years before admission. Ocular examination showed limitations on ocular motility, proptosis, inferonasal displacement of the globe, lagophthalmos, eyelid edema, and hyperemia. In contrast, Head Magnetic Resonance Imaging (MRI) revealed a mass in the left superior orbit and the right occipital condyle. Histopathological examination reveals pathological Langerhans cells with eosinophils and giant cells. The patient was treated with symptomatic drugs while chemotherapy and eyelid reconstruction were planned.
Conclusion: LCH is a disorder with highly diverse clinical manifestations. Orbital involvement is one of the uncommon presentations of LCH and necessitates a comprehensive clinical assessment. Early and accurate diagnosis is crucial. Although LCH can lead to serious complications if not promptly treated, recurrence of the disease is relatively rare. Nevertheless, long-term follow-up is recommended to ensure early detection of any potential recurrence and to maintain optimal patient outcomes.

Keywords: Langerhans cell histiocytosis, Ocular proptosis, Orbital tumor, Recurrence

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: Case report | Subject: Pediatric Hematology & Oncology
Received: 2025/01/22 | Accepted: 2025/03/8 | Published: 2025/03/30

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