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Idiopathic pulmonary haemosiderosis presenting as severe iron deficiency anaemia--a case from Pakistan

Noureen Afzal1, Ammara Mushtaq, Arshalooz Rahman

  • 1Department of Paediatrics, Aga Khan Secondary Hospital, Karimabad. noureen.afzal@aku.edu

JPMA. the Journal of the Pakistan Medical Association
|July 19, 2013
PubMed

Insights

Idiopathic pulmonary haemosiderosis is a rare lung disorder causing iron deficiency and bleeding. This case report details a child’s survival and improvement with steroids, highlighting a rare presentation of this condition.

Area of Science:

  • Pediatric Pulmonology
  • Rare Diseases
  • Hematology

Background:

  • Idiopathic pulmonary haemosiderosis (IPH) is an uncommon cause of recurrent pulmonary hemorrhage.
  • It typically presents with iron deficiency anemia, hemoptysis, and diffuse lung infiltrates.

Observation:

  • An 8-year-old child with a history of iron deficiency anemia and recurrent chest infections presented with severe respiratory distress.
  • Chest X-ray revealed bilateral opacities, and CT scans showed diffuse alveolar involvement.
  • Lung biopsy confirmed the presence of haemosiderin-laden macrophages, diagnostic of IPH.

Findings:

  • The child received steroid treatment for IPH.
  • Despite severe anemia (Hb 3.2 g/dL), the patient showed clinical improvement and survived.
  • This represents the first reported case of IPH in Pakistan.

Implications:

  • This case underscores the importance of considering IPH in children with unexplained respiratory distress and anemia.
  • Early diagnosis and prompt steroid therapy can lead to favorable outcomes in IPH.
  • Reporting this case from Pakistan expands the geographical data on this rare disorder.

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