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Critical Care: Critical Care |

Secondary Hemophagocytic Lymphohistiocytosis: A Rare Complication of Acute EBV Infection FREE TO VIEW

Sarah Robison, MD; Bhagirathbhai Dholaria, MBBS; Taimur Sher, MD; John Moss, MD
Author and Funding Information

Mayo Clinic Florida, Jacksonville Beach, FL


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(4_S):A146. doi:10.1016/j.chest.2016.02.152
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Published online

SESSION TITLE: Critical Care

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Hemophagocytic lymphohistiocytosis is a rare condition with a high mortality rate that can be difficult to diagnose because of low clinical suspicion and non-specific presenting symptoms.

CASE PRESENTATION: A 32-year-old female was admitted to the internal medicine service after presenting to the ED with myalgias, high grade fever, sore throat and a diffuse erythematous rash. Laboratory studies on admission were significant for pancytopenia and transaminitis. Several hours after admission, she became hypotensive and developed hypoxemic respiratory failure, requiring intubation and mechanical ventilation. A CT scan of the chest showed multifocal lobar consolidations and ground-glass attenuation consistent with ARDS. Empiric antibiotic therapy was started due to concern for septic shock. Infectious work-up including lumbar puncture, echocardiogram, bronchoalveolar lavage (BAL) cultures, and blood cultures were negative. Serum testing for endemic fungal infections, tick-borne illnesses, arthropod-borne illnesses, bartonella, mycoplasma, Parvovirus B19, dengue fever, Q fever, influenza/RSV, HHV6, CMV, syphilis, HIV and hepatitis were negative. However, EBV IgM and IgG were positive and serum EBV PCR measured <2000 copies/mL. Additional laboratory studies were significant for elevated ferritin level (13,664 mcg/L), hypertriglyceridemia (633 mg/dL), elevated lactate dehydrogenase (719 U/L), and elevated serum IL-2 receptors (1828 pg/mL). The patient underwent a bone marrow biopsy. The bone marrow pathology results revealed 70% cellularity with normal maturation, flow and cytogenetics. Hemophagocytosis was present. She was diagnosed with hemophagocytic lymphohistiocytosis secondary to Epstein-Barr Virus infection. Based on this new diagnosis, she was treated with 1g of IV methylprednisolone daily for 3 days, followed by a steroid taper. She improved dramatically and was extubated on hospital day 12. At the time of discharge, the pancytopenia had resolved, and serum ferritin and liver enzymes were downtrending.

DISCUSSION: Hemophagocytic lymphohistiocytosis is a non-neoplastic hematologic condition characterized by macrophage activation and cytokine release, as well as T-cell immunosuppression. Hypercytokinemia causes systemic inflammation and host tissue damage resulting in multi-organ system dysfunction. This rare condition is often underdiagnosed because patients present with non-specific symptoms such as fevers, encephalopathy, and hepatosplenomegaly. It is unfortunate that this condition is difficult to diagnose because without treatment with immune-suppressive therapy, HLH has a very high mortality rate.

CONCLUSIONS: Consider a hematologic etiology in a patient who presents with a clinical picture of sepsis, but has no clear source and is unresponsive to antimicrobial therapy.

Reference #1: Buyse S, et al. Critical care management of patients with hemophagocytic lymphohistiocytosis. Intensive Care Med (2010) 36:1695-1702.

DISCLOSURE: The following authors have nothing to disclose: Sarah Robison, Bhagirathbhai Dholaria, Taimur Sher, John Moss

No Product/Research Disclosure Information


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