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Current
Chapter:
Histiocytosis X (A.K.A. Langerhan's Cell Histiocytosis) (Updated March 2006)
Histiocytosis X (Lichtenstein 1977) or Langerhan's Cell histiocytosis (Bergholz 1979), is the currently favored term, for a spectrum of granulomatous conditions characterized by proliferating histiocytic cells admixed with eosinophils and other inflammatory cells (Dahlin 1978, Jaffe 1972, Lichtenstein 1953, McGavran 1960). Based on differences in clinical manifestations, there are four recognized variants of Histiocytosis X. Eosinophilic Granuloma (Figure 01A and 01B, Figure 01C, and Figure 01D) is the least aggressive manifestation of this group of conditions that features histiocytes as the primary proliferating cell. The other clinical syndromes include Hand–Schuller–Christian (HSC) and Letterer-Siwe (LS) disease (Lieberman 1969, Lieberman 1996), as well as the much rarer conditions, Erdheim-Chester (EC) disease and sinus histiocytosis with massive lymphadenopathy (SHML), which is also called Rosai-Dorfman (RD) disease.
The purpose of this chapter is to familiarize the reader with the spectrum of conditions encompassed under the broad designation of Histiocytosis X, with a recommended plan of management for each condition. Emphasis will be given to discussion and management of the condition most commonly referred to as Eosinophilic Granuloma.
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