Langerhans cell histiocytosis: a pathologist view

Authors: R. Kodet;  M. Mrhalová
Authors‘ workplace: Ústav patologie a molekulární medicíny 2. lékařské fakulty UK a FN Motol Praha, přednosta prof. MU Dr. Roman Kodet, CSc.
Published in: Vnitř Lék 2010; 56(Supplementum 2): 27-38
Category: Langerhans cell histiocytosis and some other Hematology rare diseases


Langerhans cell histiocytosis is a clinico- pathological entity with a wide spectrum of clinical and morphological findings. The disease was defined on the basis of recognition of three entities in medical history –  Hand- Schüller- Christian disease, Letterer- Siwe disease and eosinophilic granuloma of bone. Later on in the past, these diseases were linked under a term histiocytosis X. With identification of a common cell of origin, the Langerhans cell, a name “Langerhans cell histiocytosis” (LCH) has been accepted. This review summarizes more than a hundred-year evolution of views on the disease. Langerhans cells are identified with the aid of histopathological investigations and an ultrastructural demonstration of specific membranous intracytoplasmic structures –  Birbeck granules. At present the diagnosis of LCH utilizes immunohistochemical investigations to demonstrate positivity of S-100 protein, CD1a and langerin (CD207) in Langerhans cells. Characteristics of these molecules are briefly summarized. Further supportive laboratory methods may be used to de­monstrate expression of proteins of the cell cycle, namely Ki- 67. The evaluation of the proliferation activity might support the estimation of a potential of the disease to progress or disseminate, especially in cases diagnosed at early stages or in situations they involve a single organ or tissue, and the progression might evolve secondarily. The histopathological differential diagnosis should separate LCH from reactive non-neoplastic Langerhans cell proliferations, histocytic diseases and a number of neoplastic diseases which are briefly reviewed.

Key words:
Langerhans cell histiocytosis –  classification –  histopathological and immunophenotypical findings –  differential diagnosis


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