Histiocytosis X

Abstract

To clarify salient issues pertaining to histiocytosis X--a syndrome that includes Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma--the authors review the epidemiologic data and the histologic, morphologic, and clinical bases for diagnosis and prognosis. Histiocytes are defined and their possible histogenesis outlined, and Langerhans cells, which may be a leading element in active lesions, are characterized. The authors outline hypothetic pathogenetic schema, which they recommend be tested by recently developed immunologic and genetic means, since histiocytosis X, at least in its disseminated form, remains an unpredictable disease for which there is no proven effective therapy.

Langerhans cell histiocytosis: report of a single organ involvement in a child

“…This histological pattern is reminiscent of two autoimmune biliary diseases, primary sclerosing cholangitis and primary biliary cirrhosis. It is interesting to note that several authors have speculated, that LCH may represent a disorder/dysfunction of the immune system and not a true neoplastic process [4,5,11,12]. This hypothesis might provide an explanation for the hepatic injury seen in multi-system LCH where the liver is indirectly involved, namely that pro-and anti-inflammatory cytokines may play a role in the pathogenesis of hepatic fibrosis, even without the local presence of Langerhans cells [2,7,9].…”

“…2) Indirect involvement of liver in the absence of Langerhans cells: nonspecific portal inflammation, portal/periportal fibrosis; bile ductular proliferation; ductopenia; and eventually biliary cirrhosis [2,5,7,9,10].…”

“…The course of the disease may show extreme case-to-case variation [5]. In the majority of the cases, especially in those with localized osseous lesions, LCH is considered as a benign, reactive process [11].…”

“…granulomatous collection of histiocytes (mostly Langerhans cells) admixed with a variable number of lymphocytes, eosinophil and neutrophil granulocytes. However, some morphologic variability may occur depending on the affected tissue site and the stage of the disease [5].…”

Fine Needle Aspiration Biopsy Diagnosis of Histiocytosis-X: A Brief Review

“…Most of the cells have ovoid nuclei with prominent indentations and grooves ( Figure 3). These cells, although histiocytic in nature, lack any significant phagocytosis [3]. The relative proportion of other cell types present within these lesions varies considerably from one case to the other and occasionally from one area of the lesion to the other.…”