histiocytosis of langerhans cells (class 1 histiocytosis) consists of a range of clinical manifestations, including bone eosinophilic granuloma, hand-schüller-christian syndrome, and letterer-siwe disease. these syndromes represent a spectrum of severity and prognosis of an underlying disorder which is usually sporadic. this report describes three cases in one family, who developed the disease ...

A 54-year-old female presented with apparent isolated hypothalamic histiocytosis X associated with diabetes insipidus and Korsakoff's syndrome. Computed tomographic and magnetic resonance imaging demonstrated a single hypothalamic mass. A craniotomy for biopsy found granulation tissue of unknown cause. Further investigation discovered genital bleeding before admission. Biopsy of the cervix uter...

The lungs may be involved in patients with histiocytosis X as part of the generalised disease, but histiocytosis X confined to the lungs without extrapulmonary involvement is rare. This report describes two cases of primary pulmonary histiocytosis arising in patients with Hodgkin's disease. It is impossible to state with certainty whether the pulmonary histiocytosis arose as a response to the d...

We have investigated the secretory function of cell suspensions from bone eosinophilic granulomas surgically collected in two patients with histiocytosis X. Unseparated cell preparations spontaneously produced interleukin 1 (IL-1) and prostaglandin E2 (PGE2). In order to ascertain that this secretion was due to the characteristic Langerhans cell-like histiocytosis X cells predominantly found in...

Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease are three disorders characterized by proliferating histiocytes. Collectively, they are called histiocytosis X. Histiocytosis X often presents with lytic calvarial lesions [1]. Hypothalamic involvement is the most common intracranial manifestation [2]. Solitary histiocytosis X within the cerebral hemispheres, alth...