Langerhans cell histiocytosis in adults: a case report and review of the literature

Abstract

BackgroundLangerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children. Immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial.ObjectiveLangerhans cell histiocytosis (LCH) is easy to be misdiagnosed because of its various clinic features and laboratory results. This research focused on the clinicopathological, histopathological, immunohistochemical and other features of LCH and aimed to analyze LCH clinical features for improving diagnosis and decreasing misdiagnosis rate.Case reportA case of rare adult LCH was reported and the clinicopathological features were summarized by literature review. The multifocal form of this case includes diabetes insipidus, exophthalmos and mucocutaneous lesions in axillae and anogenital regions, such as infiltrated nodules, extensive coalescing, scaling, crusted papules and ulcerated plaques. The Langerhans cells diffusely infiltrated in the dermis and the tumor cells were positive for CD1a and S-100 expression. The diagnosis was Langerhans cell histiocytosis based on the pathological and immunohistochemical changes.ConclusionLCH has high rate of misdiagnosis and definitive diagnosis depends on pathological biopsy and X-ray examination. The prognosis is related to the onset age and the quantity of affected organs. Although specific therapeutic approach hasn't been well established, combined chemotherapy for multisystem lesions and surgical operation or radiotherapy for unifocal lesions may improve the therapy.

Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report

“…Although current experiences for the treatment of LCH suggest corticosteroids as a first choice of action, as was applied in this patient, there have been clinical studies that suggest corticosteroid therapy may be associated with a greater likelihood of deterioration than improvement [12]. The course of LCH is often difficult to predict, but in the present patient, her age, the fact that she suffered from the multi-system form of LCH, as well as having advanced cystic changes on HRCT and an active tuberculosis infection, definitely contributed to her lethal outcome [13,14].…”

Multisystemic Langerhans cell histiocytosis in an adult

“…LCH is a rare and serious disease often diagnosed late because of its manifold symptoms 3, 10. The prognosis depends on the number of organs involved, the presence of organ dysfunction, and the patient's age.…”

Adult-onset Langerhans cell histiocytosis of the sternum

“…Poor prognostic factors such as pulmonary LCH, liver function impairment, splenomegaly or bone marrow/ hematopoietic abnormity had been reported (1). Patients with MS-LCH with risk organ involvement also obtained higher mortality rate and shorten survival (1,3,4). Previous study had reported that more than 50% of investigated specimens contained a BRAF V600E gene mutation (4).…”

“…Physical examination revealed severe tenderness to touch over the sternum body and close to the manubrium. Blood hemogram revealed leukocytosis (white blood cell count: 13,140 cells/mm 3 ).…”

“…He received surgical curettage and adjuvant radiation therapy with good recovery. He is now undergoing regular out-patient (3). The definition of MS-LCH is the LCH with two or more organs/systems involvement and obtains poorer prognosis and a rapid deterioration than SS-LCH.…”