This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 6. Pulmonary Disorders Chapter 78. A rare disease of unknown etiology characterized by episodes of hemoptysis, hemorrhage into the lung, pulmonary infiltration, and secondary iron-deficiency anemia. It is most common in young children but can occur in adults. Diffuse infiltration with hemosiderin-containing macrophages is characteristic, although hemosiderin deposition occurs in many other disorders.
Pulmonary hemorrhages are most often mild and continuous but can be severe.
Blood in interstitial spaces leads to pulmonary fibrosis. Patients may live for several years, developing pulmonary fibrosis and insufficiency with chronic secondary anemia. Treatment is symptomatic and supportive. Death often results from massive pulmonary hemorrhage. All rights reserved.