A CASE OF DISSEMINATED INTRAVASCULAR LYMPHOMA PRESENTING AS SEPSIS WITH MULTIORGAN FAILURE

Abstract

TOPIC: Critical Care TYPE: Fellow Case Reports INTRODUCTION: Intravascular lymphoma (IVL) is a rare subtype of lymphoma proliferating in the lumen causing small vessel occlusion. Antemortem diagnosis is difficult. The majority of cases are diagnosed from autopsy. We report a patient with IVL who presented with sepsis of unknown source complicated by multiorgan failure. CASE PRESENTATION: A 58-year-old male with hypertension and chronic obstructive lung disease presented with fever for 3 weeks and was found to be hypotensive. Laboratory investigation was remarkable for platelet count of 84,000 per microliter, serum creatinine 3 mg/dl, aspartate aminotransferase of 50 units per liter (U/L), total bilirubin of 5 mg/dl, ferritin of 1265 microgram per liter, lactate dehydrogenase of 2051 U/L. Blood, respiratory and urine cultures were negative. CT abdomen and pelvis did not reveal a septisis source. Transthoracic echocardiogram showed a preserve ejection fraction without tamponade. Hemodynamic data from pulmonary artery catheter showed wedge pressure of 14, and mixed venous oxygen saturation of 72. He was diagnosed with septic shock with unknown source and multiorgan failure. He was treated with broad spectrum antimicrobials including Meropenem, Vancomycin and Micafungin. He was started on continuous renal replacement therapy. His clinical course continued to deteriorate. The family decided to withdraw life support. He expired on hospital day 11. An autopsy revealed intravascular large B-cell lymphoma. DISCUSSION: IVL is an extremely rare extranodal lymphoma, predominantly affecting elderly patients. The incidence remains unknown due to its rarity. The clinical presentation is variable, ranging from asymptomatic, constitutional B symptoms to multiorgan failure caused by vessel occlusion. IVL can be diagnosed by the presence of large lymphoma cells within small-to-medium blood vessels. Antemortem diagnosis of IVL is difficult due to lack of pathognomonic features or tumor markers. Symptoms could mimic other more common diseases since the clinical manifestations are heterogeneous which could lead to a delayed or missed diagnosis. IVL is aggressive and usually disseminated at the time of diagnosis. Treatment is systemic chemotherapy. CONCLUSIONS: While IVL is an extremely rare subtype of lymphoma, it should be considered in the differential diagnosis of unexplained multiorgan failure with no other clear cause. The diagnosis of IVL requires a high index of suspicion and should be suspected in patients suggestive relevant clinical findings which may lead to early treatment and better outcomes. REFERENCE #1: Bouzani M et al., "Disseminated intravascular b-cell lymphoma: clinicopathological features and outcome of three cases treated with anthracycline-based immunochemotherapy," The Oncologist, vol. 11, no. 8, pp. 923–928, 2006. REFERENCE #2: Ferreri AJ, Campo E, Seymour JF; International Extranodal Lymphoma Study Group (IELSG). Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'. Br J Haematol. 2004 Oct;127(2):173-83. REFERENCE #3: Murase T, Yamaguchi M, Suzuki R et al. Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood. 2007 Jan 15;109(2):478-85. DISCLOSURES: No relevant relationships by Maythawee Bintvihok, source=Web Response No relevant relationships by Parneet Dhaliwal, source=Web Response No relevant relationships by Joseph Guileyardo, source=Web Response No relevant relationships by Adam Hayek, source=Web Response No relevant relationships by Adan (Adam) Mora, source=Web Response