Idiopathic Pulmonary Hemosiderosis Mimicking Iron Deficiency Anemia: A Delayed Diagnosis?

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon chronic disorder in children. It is characterized by recurrent pulmonary hemorrhage and may result in hemoptysis and pulmonary insufficiency. The most common hematologic manifestation of IPH is iron deficiency anemia. The etiology of IPH is not known and its diagnosis may be difficult due to the variable clinical courses. The most helpful signs for identifying IPH are iron deficiency anemia and recurrent or chronic cough, hemoptysis, dyspnea, wheezing. We report here 5 pediatric cases of IPH presenting with iron deficiency anemia and without pulmonary symptoms. Mean corpuscular volume was low in all patients; iron was low in 4 out of 5 cases; total iron binding capacity was high in all of them; ferritin was low in 3 patients. At follow up, none of them had responded successfully to the iron therapy. Although they didn’t present with pulmonary symptoms, chest radiographs incidentally revealed diffuse reticulonoduler shadows in all of them. Computed tomography revealed diffuse ground-glass opacities, consolidation, increased density. The diagnosis was confirmed by the detection of hemosiderin-laden macrophages in bronchoalveolar lavage fluid and gastric aspirate. If patients with iron deficiency anemia don’t respond to iron therapy, they should be examined for IPH. Chest radiographs should be taken even in absence of pulmonary symptoms. Early diagnosis is important for a timely management of IPH.

Association between idiopathic pulmonary hemosiderosis and celiac disease in pediatric patients: A scoping review of the literature over the past 50 years

“…1 In contrast, pediatric patients can inadvertently swallow the sputum and may not present with hemoptysis. 57 Isolated unexplained anemia can be an unusual presentation of pediatric IPH patients. [58][59][60][61] 57% of our patients suffered from hemoptysis, which is consistent with previous pediatric literature in IPH.…”

“…Hemoptysis is present in approximately 80% of adult patients 1 . In contrast, pediatric patients can inadvertently swallow the sputum and may not present with hemoptysis 57 . Isolated unexplained anemia can be an unusual presentation of pediatric IPH patients 58–61 .…”

Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients

“…Iron deficiency anemia often occurs in malnourished children. 22 However, iron deficiency anemia is also a common manifestation of IPH, and this condition may precede other symptoms and signs by several months. 23,24 Bhatia S et al.…”

Idiopathic Pulmonary Hemosiderosis: An Unexplored Cause of Treatment Refractory Pediatric Iron Deficiency Anemia

“… 8 Overall, the prognosis is poor in children with mortality rate > 50% due to delay in diagnosis from lack of awareness of the entity and absence of classical triad. 9 The most common cause of death is an acute or chronic respiratory failure due to massive alveolar hemorrhage or cor pulmonale, respectively. 1 …”