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Pediatric Oncall Journal

Idiopathic Pulmonary Hemosiderosis in a 6 Year Old Girl 01/09/2014 00:00:00

Idiopathic Pulmonary Hemosiderosis in a 6 Year Old Girl

Asok K Datta1, Taraknath Ghosh1, Mrinal Kanti Ghosh2, Kaustav Nayak1, Sankar Kumar Das1.
1Department of Pediatrics Medicine, Burdwan Medical College, Burdwan, West Bengal, India,
2Department of Radiology, Burdwan Medical College, Burdwan, West Bengal, India.

Asok K Datta, Department of Pediatrics, Burdwan Medical College, Burdwan, West Bengal, India.
Idiopathic pulmonary hemosiderosis is an uncommon disorder, which is characterized by iron deficiency anemia, recurrent hemoptysis, diffuse parenchymal infiltration on chest radiograph. We report a six-year-old girl admitted in the pediatric emergency department with complaints of cough, respiratory difficulty, progressive pallor and bloody sputum for last two years.She had been hospitalized six times during this period and received antibiotics, bronchodilators and blood transfusion. Chest X-ray revealed bilateral pulmonary infiltrate, CT scan of lungs showed ground glass pattern, blood examination was suggestive of iron deficiency anemia and bronchoalveolar lavage yielded hemosiderin-laden macrophages. Investigations to exclude any secondary causes of hemosiderosis were negative. The child was diagnosed as Idiopathic pulmonary hemosiderosis and treated with prednisolone. Six months follow up after discharge revealed no problem.
Idiopathic pulmonary hemosiderosis is an uncommon disorder (IPH), which is characterized by recurrent hemoptysis, iron deficiency anemia and diffuse parenchymal infiltration on chest radiographs in pediatric patients [1]. Pulmonary hemosiderosis (PH) results from recurrent bleeding into alveolar spaces and interstitial lung tissues. If untreated, fibrosis and restrictive lung disease will develop and may lead to death [2]. Sometimes massive hemorrhage in the alveoli may lead to sudden death [3]. A distinction can be made between primary and secondary hemosiderosis as a manifestation of autoimmune small vessel vasculitides, coagulation disorders or diseases with increased venous pressure. Primary pulmonary hemosiderosis may occur in some children due to ingestion of cow's milk (Heiner syndrome) which usually subsided after withdrawal of milk from diet. Along with the history of hemoptysis and chest radiograph findings the presence of significant hemosiderin-loaded macrophages in bronchoalveolar lavage fluid are diagnostic of IPH. Immunosuppressive therapy has improved the prognosis in recent years [2].
Case Report
A 6 years old female child from a poor socioeconomic status family, born of a non-consanguineous marriage presented with cough, respiratory difficulty, recurrent hemoptysis and pallor with easy fatigability beginning at the age of four years. There was no history of recurrent fever, skin lesions, bleeding from any other site, arthritis, bone pain, significant drug intake or prolong exposure to toxin. Her appetite, bladder & bowel habits were normal. She had received antibiotics, bronchodilators treatment along with iron prophylaxis and total six blood transfusions. At the time of admission, the child had marked pallor, respiratory distress, tachypnea (respiratory rate = 46/min) and tachycardia (pulse = 120/min). Other vital parameters were normal. Her weight was 15 kg and height was 105.5 cm. Examination of chest revealed crepitations bilaterally. Other examination, findings were normal. On investigation, hemoglobin was 4.6gm% with hypochromic microcytic anemia, total leukocyte count was 8700/cu mm (polymorph 58%, lymphocyte 30%, eosinophil 07%, monocyte 03%), platelets were adequate, serum ferritin level was low (4mg/l), total iron binding capacity was 500mg/dl(Normal = 250-400 mg/dl) suggestive of iron deficiency anemia. Clotting time, prothombin time, activated partial thromboplastin time, liver function test, renal function test were normal. Antinuclear antibody (ANA), anti-neutrophil cytoplasmic antibody (ANCA) and Mantoux test were negative. Chest radiograph revealed bilateral pulmonary infiltrate (Figure 1). CT scan showed bilateral ground glass opacity (Figure 2). Bronchoalveolar lavage demonstrated significant number of hemosiderin-loaded macrophages (Figure 3). As no cause was identified, the child was diagnosed as idiopathic pulmonary hemosiderosis. The patient was given blood transfusion and oral prednisolone in the dose of 2 mg/kg along with iron. Her condition improved after 7 days of therapy. After 1 month of treatment, chest radiograph was clear and there was no further hemoptysis for last 6 months.

Figure 1: Chest X-ray shows bilateral pulmonary infiltrate
<b>Figure 1: Chest X-ray shows bilateral pulmonary infiltrate</b>

Figure 2: CT scan shows bilateral ground glass opacity
<b>Figure 2: CT scan shows bilateral ground glass opacity</b>

Figure 3: Bronchoalveolar lavage demonstrates significant number of hemosiderin laden macrophages.
<b>Figure 3: Bronchoalveolar lavage demonstrates significant number of hemosiderin laden macrophages. </b>
Idiopathic pulmonary hemosiderosis is a rare disease of unknown etiology characterized by the triad of hemoptysis, pulmonary infiltrate on chest radiograph and iron deficiency anemia [4,5]. Our patient presented with similar findings of cough, recurrent hemoptysis along with features suggestive of iron deficiency anemia. She also had bilateral lung infiltrates. The diagnosis is based on the presence of iron deficiency anemia, characteristic chest radiograph and demonstration of hemosiderin-laden macrophages in bronchoalveolar lavage [6] as was done in our patient. Secondary causes of hemosiderosis are to be excluded. We have extensively searched for secondary cause but did not any. Corticosteroids and/or immunosuppressive drugs may be effective during acute bleeding episode and may in some patients improve symptoms and prognosis in the long term [7]. Our patient responded with prednisolone and there have been no recurrence in last six months follow up.

Competing interests:
The authors declare that they have no competing interest.
Authors Contribution
AKD was responsible for patient care, follow up and drafting of paper, TNG and KN collected data and SD assisted AKD to prepare manuscript. MKG was involved in the radiological investigation of the study. All authors read and approved the final manuscript
We acknowledge Dr.Subodh Kumar Bhattacharya, the Head of Department of Pathology for his active co-operation.
Compliance with Ethical Standards
Funding None
Conflict of Interest None
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Cite this article as:
Datta A K, Ghosh T, Ghosh M K, Nayak K, Das S K. Idiopathic pulmonary hemosiderosis in a 6 years old girl. Pediatr Oncall J. 2009;6: 17.
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