SESSION TITLE: Lung Cancer Cases - Student/Resident
SESSION TYPE: Student/Resident Case Report Slide
PRESENTED ON: Sunday, October 25, 2015 at 03:15 PM - 04:15 PM
INTRODUCTION: Intravascular Lymphoma (IVL) is a rare malignancy that is historically difficult to diagnose and is frequently identified post mortem. The illusive nature of IVL stems from its ability to grow in the intima of capillaries and migrate allowing for easy spread and frequent false negative biopsies. Pulmonary presentations of IVL have been described in the literature, including: interstitial lung disease, aspergillosis, pulmonary embolism, small bilateral nodules, and large lung nodules. We describe a case of IVL presenting with recurrent fevers and a 2cm left upper lobe nodule complicated by diffuse alveolar hemorrhage (DAH).
CASE PRESENTATION: A 54 year old presented with a 2 month history of recurrent fevers, ataxia, and fatigue. The patient noted a previous episode 7 years ago characterized by splenomegaly and thrombocytopenia and which resolved without a formal diagnosis. His examination was remarkable for a temperature of 38°C and splenomegaly without lymphadenopathy. His laboratory studies demonstrated a leukocyte count of 2,400/mcl, hemoglobin of 8.7g/dL, and platelet count of 30,000/mcl. His LDH was 1054IU/L. His bone marrow biopsy was normal. CT of the chest, abdomen, and pelvis demonstrated an irregular 2cm x 1cm left upper lobe opacity which was located at the lateral periphery. Subsequent CT guided lung biopsy was complicated by a left sided hemothorax. He underwent emergent thoracotomy with a left upper lobe wedge resection. This biopsy yielded a diagnosis of IVL. On day 15 postoperatively, he developed hypoxemic respiratory failure and required endotracheal intubation. Bronchoscopy showed diffuse bleeding in the lungs and chest X-ray changes were consistent with acute onset DAH. Unfortunately the patient deteriorated and died from profound thrombocytopenia and DAH.
DISCUSSION: Originally described in 1959 as angioendotheliomatosis proliferans systemisata (APS), IVL is now well described with multiple references to pulmonary presentations. As of 2001, the World Health Organization (WHO) classified IVL as a subtype of diffuse large B-cell lymphoma (1). There have however been cases of T-cell IVL and these cases frequently involve the skin. There is no standard method in the diagnosis of IVL though random skin biopsies have been shown to be useful. Landmarks in the treatment of IVL include the discovery of the monoclonal antibody rituximab. To date, this is the second case of IVL with associated DAH.
CONCLUSIONS: In patients with aggressive pancytopenia, elevated LDH, splenomegaly, and the absence of a detectable tumor, a diagnosis of IVL should be considered. A delay in timely diagnosis often results in death, and the prognosis of IVL is extremely poor. Better recognition of IVL is essential for prompt treatment with rituximab based chemotherapy steroids to obtain optimal remission and survival rates.
Reference #1: 1. Primary intravascular large B-cell lymphoma of lung: a report of one case and review. Yu H, Chen G, Zhang R, Jin X. Diagn Pathol. 2012
DISCLOSURE: The following authors have nothing to disclose: Allan Bates, Jordan Ray, Gaja Shaughnessy, Jennifer Hsu, John Moss, Halawa Ahmad
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