Jornal de Citologia e Histologia

Clinical Features and Management of Langerhans Cell Histiocytosis: A Case Series

Abstract

Tayssir Ben Achour1*, Maysam Jridi, Ines Naceur, Monia Smiti, Fatma Said, Mohamed Habib Houman and Eya Azouz

Background: Langerhans histiocytosis (LCH) is a rare hematopoietic disease characterized by clonal expansion of myeloid precursors differentiating into CD1a+/CD207+ histiocytes infiltrating different organs. The study aimed to report the clinical features such as the onset presentation and systemic lesions of LCH among Tunisian adult patients and the therapeutic management of the disease with onboard medications.

Methods: A descriptive, retrospective and monocentric study taking place in the internal medicine department of Rabta University Centre.

Results: The study included eight patients. The median age at the diagnosis was 35 years. The mean duration between the first symptoms and the diagnosis was 17 months. The first most consulted doctors were neurologists. The revealing symptoms were neurological in five cases including headaches, paraplegia or walking trouble. Bone pain was the first symptom in three cases and in one case cervical adenopathy was the first manifestation. Bone involvement was the most frequently found (N=6). Four patients had central nervous system lesions. PLCH was observed in four cases and only one patient was a smoker. Two patients had lymphadenopathy. Retroperitoneal fibrosis and abdominal aortitis were found in one case. Skin involvement was found in one case and was associated with a multisystem LCH form. We observed one case of orbital involvement with optical canal infiltration. In all cases, the diagnosis of LCH was established based on histological features. Seven patients received a treatment with prednisone. Vinblastine was used in six cases.

Conclusion: Langerhans cell histiocytosis is a rare disease with different facets and heterogenous initial presentation. Patients with LCH would usually see different doctors and would have misleading signs with a variety of differential diagnoses before recognizing LCH.