SESSION TITLE: Lung Pathology Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Anti-synthetase syndrome is characterized by the presence of anti-Jo 1 antibodies in conjunction with clinical findings of fever, polymyositis-dermatomyositis and interstitial lung disease (ILD). Inflammatory myopathies are associated with an increased risk of malignancy, but this association is less well-outlined for patients with anti-synthetase syndrome. In fact, reports suggest the presence anti-Jo1 antibodies are protective against malignancy. We present a rare case of peripheral T-cell lymphoma presenting as hypoxemic respiratory failure in a patient with anti-synthetase syndrome
CASE PRESENTATION: A 44-year old female with anti-synthetase syndrome presented with progressively worsening dyspnea, a dry cough and developed acute hypoxemic respiratory failure. She was empirically treated with broad spectrum antibiotics for presumed health-care associated pneumonia; however, her clinical condition did not improve. Chest computed tomography (CT) revealed extensive bilateral ground glass opacities and axillary and mediastinal lymphadenopathy. Infectious work-up was negative. Surgical lung biopsy revealed peripheral T-cell lymphoma (PTCL). The patient was started on combination cyclophosphamide, doxorubicin, vincristine, and prednisone. Two days later, both hypoxemia and bilateral infiltrates resolved and the patient was successfully extubated.
DISCUSSION: Our case illustrates a unique presentation of PTCL in a patient with anti-synthetase syndrome. First, lung involvement in PTCL is exceedingly rare (8%)  and can be diagnostically challenging. Few case reports document lung involvement in PTCL in the form of a ‘crazy-paving pattern’ and ‘pulmonary nodules’ ; however, none of these patients presented with acute hypoxemic respiratory failure nor had anti-synthetase syndrome. Second, contrary to prior reports, anti-Jo 1 antibodies were not protective against malignancy in our patient, thus highlighting an important dissociation.
CONCLUSIONS: To our knowledge, ours is the first case of PTCL presenting in a patient with anti-synthetase syndrome as hypoxemic respiratory failure with bilateral multifocal lung consolidation.
Reference #1: Weisenburger, D.D., et al., Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood, 2011.
Reference #2: Vahid, B., Pulmonary manifestations of peripheral T-cell lymphoma: case report and review of the literature. Clin Respir J, 2007.
DISCLOSURE: The following authors have nothing to disclose: Masooma Aqeel, Jayshil Patel
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