C62-28 Idiopathic Pulmonary Hemosiderosis Masquerading as Hemolytic Anemia in a Toddler

Introduction Idiopathic pulmonary hemosiderosis (IPH) is characterized by recurrent diffuse alveolar hemorrhage that leads to hemoptysis, iron deficiency anemia, and diffuse pulmonary infiltrates. This classic triad is often incomplete in pediatric patients resulting in frequent delays in diagnosis. We present a case of a young child who presented with hemolytic anemia and was ultimately found to have IPH. Case description An 18-month-old male with a history of iron deficiency anemia and multiple presentations of hemolytic anemia in the setting of viral infections presented to the pediatrician with increased work of breathing, fatigue, decreased appetite, and 5-day history of pallor. Point-of-care hemoglobin was notably low at the PCP’s office. Family was directed to the ED where he was tachycardic and hypoxemic requiring oxygen via nasal cannula. Chest x-ray revealed diffuse hazy opacification within bilateral lungs. Respiratory viral PCR panel was negative. Initial CBC revealed WBC 14.3 (5.3-13.2 10*9/L), Hb 7.0 g/dl (9.9-13.4 g/dl), HCT 20.8% (30.0-40.0%), reticulocyte count 11.0% (0.66-2.55%), LDH 914 U/L (226-373 U/L), Haptoglobin <10 (30-200mg/dl), direct bilirubin 0.50 (0-0.30 mg/dL) and total bilirubin 1.5 (0.3-1.2 mg/dL). He received 10ml/kg packed red blood cell transfusion and was admitted to hematology service. On day 4, flexible bronchoscopy revealed normal airways and grossly bloody return upon bronchoalveolar. Cytology of BALF was remarkable for numerous RBCs and extensive hemosiderin deposition indicative of old hemorrhage on iron stain. BALF was negative for bacterial culture, and viral and pneumocystis PCR testing. Autoimmune workup was negative including C3 and C4, ANA, anti-dsDNA, ANCA, anti-GBM, anti-phospholipid panel. On day 8, he underwent video-assisted thoracoscopic surgery with wedge resections of the right upper and right middle lobes. Surgical pathology was remarkable for diffuse intra-alveolar hemorrhage with pigmented macrophages containing hemosiderin. There was no evidence of vasculitis or capillaritis. He received 3 days of methylprednisolone 30mg/kg and was subsequently discharged on a prednisolone taper for 3 months. Discussion The etiology of hemolytic anemia in this patient is unknown, and workup is ongoing including whole exome sequencing. This case is interesting as it presents a case of concurrent hemolytic anemia and IPH. IPH is rare and requires exclusion of other causes of pulmonary hemorrhage such as vasculitis or capillaritis. In this case, lung biopsy was positive for IPH and there were no signs of hemolysis. Early diagnosis of IPH is important in preventing permanent lung injury by initiating corticosteroids to optimize prognosis in children.