Clinical case of idiopathic pulmonary hemosiderosis in a young child: Diagnostic and therapeutic challenges

Idiopathic pulmonary hemosiderosis (IPH) is an orphan interstitial vascular lung disease that primarily affects children. The condition is characterized by a classic triad of symptoms: anemia, hemoptysis, and pulmonary consolidation visible on chest radiography and computed tomography.

Objective. To analyze the clinical and diagnostic features and therapeutic strategy for treatment-refractory IPH in a young child based on a detailed review of the fatal case.

Materials and methods. A clinical case of a 1‑year‑10‑monthold patient with confirmed IPH is presented, including the stages of treatment in the pulmonology department and the intensive care unit. The diagnosis was established based on a combination of progressive respiratory failure, characteristic findings on chest computed tomography, and the presence of a pathognomonic cytological sign, i. e., siderophages in bronchoalveolar lavage fluid, and following exclusion of Heiner’s syndrome and other causes of pulmonary hemosiderosis. The selected therapy included respiratory support and immunosuppressive treatment.

Results. The case demonstrated progressive disease refractory to combined immunosuppressive therapy. Despite comprehensive treatment, the patient developed life-threatening complications (severe acute respiratory distress syndrome, air leak syndrome, and multiple organ failure), which led to a fatal outcome.

Conclusion. The presented case illustrates an extremely unfavorable course of IPH in young children, underscoring the need for early diagnosis, readiness for immediate initiation of intensive therapy, and the search for new effective treatment protocols for severe forms of IPH.

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