Diagnostic evaluation of patients with histiocytosis X

Abstract

The natural history of histiocytosis X was reviewed in 52 patients to identify the characteristics of patients with localized versus disseminated disease. Two categories of patients were identified. Older patients with solitary or multiple bone lesions constituted a low-risk group. This group had a high rate of development of new bone lesions (55% were asymptomatic) but an excellent prognosis. Patients with soft tissue involvement constituted a group at high risk for disease progression and organ dysfunction. These patients required a more extensive diagnostic workup and systemic treatment. A diagnostic algorithm is proposed to identify these two groups.