Abstract
Acute fibrinous and organizing pneumonia (AFOP) is an extremely rare, relatively new, and distinct histological pattern of acute lung injury characterized predominately by the presence of intra-alveolar fibrin and associated organizing pneumonia. AFOP may be idiopathic or associated with a wide spectrum of clinical conditions. It has a variable clinical presentation from mild respiratory symptoms to that similar to the acute respiratory distress syndrome. Currently there is no consensus on treatment, and corticosteroids previously were of unclear benefit. To date, there are less than 40 cases of AFOP reported in the literature and only one has been linked to hematopoietic stem cell transplantation. Here we report the first case series of 2 patients who developed AFOP following allogenic stem cell transplant that were successfully treated with high-dose corticosteroids.
Highlights
Acute fibrinous and organizing pneumonia (AFOP) is an extremely rare diffuse parenchymal lung disease that was first described by Beasley et al[1] as a possible variant of diffuse alveolar damage (DAD), bronchiolitis obliterans with organizing pneumonia (BOOP), or eosinophilic pneumonia (EP)
acute fibrinous and organizing pneumonia (AFOP) has been observed in patients with collagen vascular disease, various infections, occupational exposures, or adverse drug reactions,[1,2] making it difficult to identify clinical characteristics that are specific to AFOP
We report the first case series of 2 patients who developed AFOP following allogenic hematopoietic stem cell transplant (HSCT), which suggests AFOP may be more common in this patient group than previously recognized
Summary
Acute fibrinous and organizing pneumonia (AFOP) is an extremely rare diffuse parenchymal lung disease that was first described by Beasley et al[1] as a possible variant of diffuse alveolar damage (DAD), bronchiolitis obliterans with organizing pneumonia (BOOP), or eosinophilic pneumonia (EP). Despite administration of broad-spectrum antibiotics and antifungals, the patient had worsening cough, dyspnea, and progressive hypoxemia, and underwent surgical lung biopsy on hospital day 9 with pathology consistent with AFOP (Figure 4). A 25-year-old Hispanic man presented 25 days after allogenic HSCT for relapsed B-cell lymphoma with persistent fevers of 102°F, progressive dyspnea, and cough. He underwent bronchoscopy with BAL of the left lower lobe; WBC count was 970/mL (4% polymorphonuclear cells, 41% lymphocytes, 38% histiocytes, and 17% macrophages).
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