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Severe iron deficiency anemia in a child with idiopathic pulmonary hemosiderosis: a case report
Murat Derbent1, Figen Ozçay, Umit Saatçi
1Department of Pediatrics, Başkent University, Faculty of Medicine, Ankara, Turkey.
Insights
Idiopathic pulmonary hemosiderosis (IPH) can cause severe iron deficiency anemia. High-dose, short-term corticosteroid treatment effectively managed acute respiratory hemorrhage in a pediatric case unresponsive to standard doses.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Rare Diseases
Background:
- Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of recurrent pulmonary hemorrhage.
- Iron deficiency anemia (IDA) is a common condition in children, but IPH is an uncommon etiology.
- Delayed diagnosis of IPH can lead to significant morbidity, including unnecessary treatments and transfusions.
Observation:
- A 3-year-old male presented with severe IDA, initially diagnosed and treated with multiple blood transfusions without identifying the underlying cause.
- Lung biopsy revealed hemosiderin-laden macrophages, confirming the diagnosis of IPH.
- The patient showed no response to standard-dose corticosteroid (CS) therapy (2 mg/kg/d).
Findings:
- High-dose, short-term CS treatment successfully managed two episodes of acute respiratory hemorrhage in this IPH patient.
- Standard-dose CS treatment was ineffective for this patient's IPH management.
- The case highlights the diagnostic challenge of IPH presenting solely as severe IDA.
Implications:
- IPH should be considered in the differential diagnosis of unexplained severe iron deficiency anemia in children.
- Prompt diagnosis of IPH can prevent extensive investigations and unnecessary blood transfusions.
- High-dose, short-term CS therapy may be a viable treatment option for acute respiratory hemorrhage in IPH patients refractory to standard doses.
Abstract:
We report a case of idiopathic pulmonary hemosiderosis (IPH) in a three-year-old male patient who presented with severe iron deficiency anemia. The child had been diagnosed with iron deficiency anemia nine months earlier and had received multiple blood transfusions, but the cause of his anemia had not been established. The diagnosis of IPH was made after a biopsy of the left lung showed large numbers of hemosiderin-filled macrophages in the alveoli. He did not respond to standard dose corticosteroid (CS) treatment (2 mg/kg/d). However, high-dose short-term CS treatment was successful in two episodes of acute respiratory hemorrhage in this patient. We conclude that IPH should always be considered when investigating the cause of iron deficiency anemia. A more rapid diagnosis in this case could have prevented unnecessary investigations and blood transfusions. We also suggest that high-dose short-term CS treatment should be kept in mind, especially in patients who do not respond to a standard dose.
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