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A3394 - A Troubling Trifecta! Case of HIV, HLH and Hodgkin’s Lymphoma
Author Block: K. Vijaykumar, P. Mathur, A. Bansal; Rochester General Hospital, Rochester, NY, United States.
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a potentially devastating hyper-inflammatory condition often complicated with sepsis. We present a case of HLH secondary to Epstein-Barr viremia in an HIV positive individual, incidentally found to have Hodgkin’s lymphoma. Case: A 50-year-old lady, HIV positive since 1987, non-compliant on HAART, with significant history of meningitis and esophageal candidiasis was admitted with fever and malaise of one month’s duration. She was found to be confused, febrile (101.4F), tachycardic (102/min) and hypotensive (93/56mmHg). Examination revealed scleral icterus and oral ulcers. Laboratory values were significant for lactic acidosis (2.8mmol/L) without leukocytosis and high INR (2.2). She was admitted to intensive care unit for the need of vasopressor support and started on intravenous vancomycin and piperacillin/tazobactam for septic shock. Given her worsening encephalopathy and severely immunocompromised status (CD4 count 51), antibiotic therapy was broadened to include trimethoprim-sulfamethoxazole and acyclovir to cover for opportunistic infections. Spinal tap was unrevealing for infection; CT head was negative for evidence of HSV encephalopathy or toxoplasmosis and MRI brain showed symmetric confluent zones of abnormal areas throughout the periventricular white matter suggestive of HIV encephalitis. Multiple blood and urine cultures remained unrevealing for any source of infection; serologies for histoplasma, toxoplasma, parvovirus B19, and cryptococcus were negative. The patient continued to deteriorate with ensuing respiratory failure and required to be intubated. Alternate diagnoses were explored- she tested positive for elevated levels of EBV DNA. CT imaging of the abdomen showed hypodense lesions in the liver, hepatosplenomegaly and paraaortic lymphadenopathy. Bone marrow biopsy was obtained with a high suspicion of lymphoma and revealed histiocytes containing phagocytosed hematopoietic cells diagnostic of HLH, presumably secondary to EBV viremia. Patient was started on HLH-94 protocol with etoposide and decadron. She worsened despite therapy with ensuing kidney failure and died following cardiac arrest. Autopsy report revealed HLH and evidence of Hodgkin’s lymphoma on the bone marrow.Discussion: This is a case of HLH presumed secondary to EBV viremia in the setting of HIV and Hodgkin’s lymphoma. HLH is a condition with variety of clinical findings and a challenging diagnosis with high mortality and morbidity. Immunocompromised patients presenting with persistent fevers typically prompt workup for disseminated infections, but conditions like HLH should be an early differential since aggressive treatment leads to reduction in associated mortality.