Histiocytosis X

Abstract

1. The clinical, histological and radiological findings in 129 cases of histiocytosis X have been reviewed and the natural history of the disease is discussed. 2. Certain clinical and histological factors emerge as having prognostic significance. 3. The treatment in thirty-four personally reviewed cases is discussed and some suggestions are made as to the treatment of particular types of case.

Langerhans’ cell histiocytosis of the spine in children with soft tissue extension and chemotherapy

“…Langerhans' cell histiocytosis (LCH) is a rare disease associated with a proliferation of Langerhans' cells [1,6] and encompasses three disparate diagnoses: Letterer-Siwe disease, Hand-Schüller-Christian disease and eosinophilic granuloma (EG) [7]. Up to 80% of LCH lesions in children are of EG type [2].…”

Solitary eosinophilic granuloma of sternum: case report with review of the literature

“…Some authors cite involvement of the skull and femur in 50% of patients below 20 years of age, with predominant involvement of ribs and mandible in the over-20 age-group (Mickelson & Bonfiglio, 1977;Cohen et al, 1980). Others (Fowles & Bobechik, 1970;Cheyne, 1971) found skull lesions in 50% of cases and femur plus pelvis in 25%. Findings of other authors reinforce the evidence that the most common sites are the skull and jaws (Dargeon, 1965;Lily & Marlett, 1970;Stern et al, 1976;Granada & McDaniel, 1982).…”

“…The general consensus in the majority of the articles published is that EG is a destructive lesion of bone representing the localised manifestation of histiocytosis X and comprises approximately 50%-80% of histiocytosis-X cases (Mickelson & Bonfiglio, 1977;Cohen et al, 1980). The lesion is seen predominantly in childhood (Arcomano et al, 1961;Sbarbaro & Francis, 1961;Ochsner, 1966 1969;Cheyne, 1971;Lahey, 1975;Sims, 1977) and in Caucasian patients, although several cases of elderly patients and coloured patients have been reported. The reported male to female incidence ratio is 2 : 1 (Oberman, 1961;Liberman et al, 1969;Cohen et al, 1980;McCullough, 1980).…”

Eosinophilic Granuloma of Bone

“…In all reported cases of children with neurological complications of vertebral lesions, neurological recovery occurred and the vertebral lesions healed. In adults, spinal cord damage may occur with either multiple or solitary vertebral lesions (Oberman 1961, Oschner 1966, Cheyne 1971 would also like to thank Dr. J. L. Boldero for allowing access to his X-ray collections.…”