Arrecillas ZMD, Ortiz HC, Uribe UN, Chávez ML, Olvera RJE

Language: Spanish
References: 17
Page: s26-s30
PDF size: 453.18 Kb.

ABSTRACT

The Langerhans cell histiocytosis (LCH) is a clonal proliferation of antigen presenting dendritic cells with three clinical forms according to the affected sites. Rosai-Dorfman’s disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a reactive process with a characteristic clinical picture and policlonal histiocytic proliferation. In both diseases the diagnosis is made microscopically, the key features are in the former the Langerhans cells and in the latter, the emperipolesis. They have characteristic immunohistochemical and ultrastructural features. This paper presents the case of a 42-year-old woman, with history of long standing diabetes mellitus. Bilateral frontoparietal and right occipital, nodular destructive lesions were described in neuroimaging studies. In a lymph node biopsy the diagnosis of Langerhans cell histiocytosis with probable association with Rosai-Dorfman’s disease was made. Seven days prior to her demise patient developed a mass in the right lateral aspect of the neck. On the day before her death her neurological symptoms worsened and died in a state of coma. The autopsy findings were extensive, destructive lesions by an orange yellow neoplasm that infiltrated the neighboring soft tissues, skin and occipital duramatter. The cervical lymph nodes as well as the peripancreatic and pericolic were grossly affected. In all these sites the characteristic microscopic features of both diseases were easily identified. The ultrastructural and immunohistochemical studies confirmed the diagnoses. The positivity for CD1a in the histiocytes of LCH was a definitive finding. These two diseases are uncommon and their association is exceedingly rare. Only one case was found in the literature. Both diseases can mimic meningeal or intraaxial neoplasms. The clear distinction of the two is paramount since the clinical behavior, the prognosis and treatment are different.

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