BURKITT LYMPHOMA MIMICKING SEPTIC SHOCK: AN UNUSUAL PRESENTATION

Abstract

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Burkitt lymphoma (BL) is a highly aggressive non-Hodgkin B-cell lymphoma that typically presents with multiple metabolic derangements, kidney failure, involvement of the jaw or other facial bones, and enlarged lymph nodes. Our case report describes an initial presentation of (BL) with septic shock. CASE PRESENTATION: 25-year-old Caucasian female presented with a 4-day history of sharp non-radiating right upper quadrant abdominal pain, poor appetite, nausea, multiple episodes of loose watery stools, and polydipsia. Exam was notable for 2/4 SIRS criteria, with heart rate 134 and respiratory rate 29, and right upper quadrant was tender to palpation. Workup revealed white blood cell count 10.3 thou/uL, lactate 4.5 mmol/L, total bilirubin 1.5 mg/dL with direct bilirubin 0.8 mg/dL, AST 138 U/L, ALT 177 U/L, and alkaline phosphatase 601 U/L. Despite a 30cc/kg fluid bolus for resuscitation, lactate worsened to 5.2 mmol/L. With concern for septic shock, the patient was started on broad-spectrum antibiotics and admitted to the hospital with an intra-abdominal source suspected. Computed Tomography (CT) scan of the abdomen and pelvis with contrast demonstrated numerous hypoenhancing lesions of the liver (up to 80% of parenchyma), spleen, right kidney, and right adrenal gland. Blood cultures remained negative, with antibiotics discontinued after 48 hours, and lactate clearing with further fluid resuscitation. The patient underwent biopsy of the liver, which revealed findings consistent with Burkitt lymphoma (CD10, 19, 20 + on flow cytometry; Myc+, bcl2 and 6 – on FISH. PAX5, BCL6, and CD10 + and bcl2 - on immunochemistry). With improvement in symptoms, the patient was discharged with oncology follow-up. DISCUSSION: BL is a rare, but very aggressive, B-cell non-Hodgkin lymphoma, often associated with Ebstein Barr virus, human immunodeficiency virus, and overexpression of oncogene C-MYC on chromosome 8. It presents in three definite forms: endemic, sporadic, immunodeficiency-associated[4]. The sporadic variant is more common among caucasians[3] and in patients below 35 years of age with a median age at diagnosis of 30 years[1,2]. Patients usually present with abdominal signs and symptoms involving the small and large intestine mimicking acute appendicitis or intussusception. Excisional biopsy is preferred for the diagnosis of BL, with confirmation done by immunohistochemistry and cytogenetics. While aggressive, it is highly curable with a two-year survival rate of 80-90%, treatment consisting of multi-agent regimens. CONCLUSIONS: Clinicians, especially intensivists, should be aware of the numerous clinical conditions, such as Burkitt Lymphoma, which may mimic sepsis and septic shock as early diagnosis can improve outcomes and decrease overuse of antibiotics. Diagnosing Burkitt Lymphoma early can also improve patients' mortality with the initiation of early treatment to optimize survival. REFERENCE #1: World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC Press, Lyon 2008. REFERENCE #2: Armitage JO, Weisenburger DD ., New approach to classifying non-Hodgkin's lymphomas: clinical features of the major histologic subtypes. Non-Hodgkin's Lymphoma Classification Project., J Clin Oncol. 1998;16(8):2780. REFERENCE #3: Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS., Lymphoma incidence patterns by WHO subtype in the United States, 1992-200., Blood. 2006;107(1):265. Epub 2005 Sep 8. DISCLOSURES: No relevant relationships by Bohdan Baralo, source=Web Response No relevant relationships by Shambo Guha Roy, source=Web Response No relevant relationships by SANA MULLA, source=Web Response No relevant relationships by Ruqqiya Mustaqeem, source=Web Response No relevant relationships by Monisha Panda, source=Web Response No relevant relationships by Mahati Paravathaneni, source=Web Response No relevant relationships by Rajesh Thirumaran, source=Web Response No relevant relationships by Vihitha Thota, source=Web Response