IDIOPATHIC PULMONARY HEMOSIDEROSIS IN CHILDREN: A ROMANIAN EXPERIENCE

Abstract

INTRODUCTION: Idiopathic pulmonary hemosiderosis (IPH) is a rare disease with unknown cause and variable outcome. It is characterized by recurrent episodes of severe hypochromic anemia, alveolar bleeding, and typical radiologic findings. OBJECTIVE: The objective of this study was to develop an early diagnosis of IPH with real therapeutic benefits. METHODS: We conducted a multicenter, retrospective, and prospective study using patients who were admitted to 3 Romanian pediatric clinics between 1984 and 2006. Secondary causes of pulmonary hemosiderosis were excluded. RESULTS: Fifteen patients received a diagnosis of IPH during a 22-year period (1984–2006). The symptoms started at a mean age of 6.8 years (range: 9 months to 13 years), with a mean delay of 2.4 years before diagnosis. From the beginning, all patients had anemia, and only 6 children presented with pulmonary symptoms as well. The classical triad (anemia, hemoptysis, and pulmonary infiltrates) was found from early in the disease in only 4 patients. The majority of patients' disease was diagnosed by bronchoalveolar lavage, and 3 were diagnosed at necropsy. Eight patients died in a period of 1 to 3 years from the diagnosis. The clinical course was variable: treatment with corticosteroids alone was not effective because 12 patients continued to have recurrent bleeding. Three patients who received immunosuppressive agents had a better outcome. CONCLUSIONS: IPH is a severe condition with variable prognosis and has a better outcome when diagnosis is made at an early age. We believe that it is necessary to include in the screening of any severe, recurrent, hypochromic anemia a well-interpreted chest radiograph and to look for hemosiderin-laden phages in bronchoalveolar lavage.