View Abstract

A3389 - Multi-Organ Failure with Hemophagocytic Lymphohistiocytosis Due to Coexistent Ehrlichia and Epstein-Barr Virus Infections
Author Block: S. Patel1, P. Denoux2, A. Manohar3, T. Broeseker4, A. L. Fernandez5; 1Internal Medicine Residency Program, The Florida State University College of Medicine at Tallahassee Memorial Hospital, Tallahassee, FL, United States, 2Clinical Sciences, The Florida State University College of Medicine at Tallahassee Memorial Hospital, Tallahassee, FL, United States, 3Southeastern Center for Infectious Diseases, Tallahassee, FL, United States, 4Tallahassee Memorial Physician Partners Cancer and Hematology Specialists, Tallahassee, FL, United States, 5Tallahassee Pulmonary Clinic, Tallahassee, FL, United States.
INTRODUCTION
Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome distinguished by uncontrolled phagocyte activation leading to a life threatening hyper-inflammatory response typically affecting pediatric patients. Secondary HLH in adults is often triggered by infections and malignancy. Ehrlichiosis is a tick-borne disease prevalent in southeastern United States and rarely can cause HLH. We present a case of multi-organ failure with HLH secondary to Ehrlichia chaffeensis and Epstein-Barr virus (EBV) infections.
PRESENTATION
A 62-year-old male with no medical history presents with two weeks of flu-like symptoms with non-productive cough, sore throat, rhinorrhea, malaise, myalgias, night sweats, and fevers. He reported relief of his sore throat and rhinorrhea with over-the-counter medications but his fever persisted. He was subsequently placed on an antibiotic by his primary care physician but had no improvement over the next three days. His symptoms progressed to lethargy, new onset right flank pain, nausea, vomiting and constant non-bloody diarrhea daily for which the patient was evaluated at our tertiary medical center. Vital signs were significant for temperature of 39.1 degrees Celsius, 30 breaths per minute, 122 beats per minute, and blood pressure of 94/59 mmHg with oxygen saturation of 91% on two liters nasal cannula. Physical examination was significant for irregularly irregular tachycardia, diaphoresis, pallor with clear lungs and no defining rash. Initial lab work was concerning for pancytopenia, kidney and liver failure, elevated CPK level, and severe hyperferritinemia requiring transfer to intensive care unit, initiation of broad spectrum antibiotics and emergent hemodialysis. Chest X-ray showed left lower lobe plate-like atelectasis. Renal ultrasound noted incidental splenomegaly. Upon further review of history, it was discovered that he had no recent travel, sick contacts, contaminated food sources, tick bites or rodent exposure. Patient did report living in a north Florida suburb adjacent to a wildlife area with a substantial white-tail deer population. Diagnostic workup revealed positive EBV and Ehrlichia chaffeensis by polymerase chain reaction, positive Mycoplasma pneumoniae serology and bone marrow biopsy was consistent with HLH. Patient was started on etoposide and dexamethasone with improvement in symptoms, organ function, and ferritin levels.
CONCLUSION
HLH is a rare hyperstimulated immune response associated with multi-organ failure requiring a multidisciplinary approach and immediate recognition for appropriate treatment. Our case is distinct with coexistent infections including Ehrlichia chaffeensis and EBV in an endemic area for white-tailed deer. Although HLH is often lethal, the prognosis of infection-induced HLH is excellent with prompt diagnosis and initiation of treatment.