Liver Abscesses with Bacteria-Associated Hemophagocytic Syndrome

Case Report

Ann Hematol Oncol. 2015; 2(9): 1064.

Liver Abscesses with Bacteria-Associated Hemophagocytic Syndrome

Takamatsu A¹*, Nagai Y² and Constantine SH¹

¹Department of General Internal Medicine, Teine Keijinkai Hospital, Japan

²Department of Hematology, Teine Keijinkai Hospital, Japan

*Corresponding author: Akane Takamatsu, Department of General Internal Medicine, Teine Keijinkai Hospital, 1-12-1-40, Maeda, Teine-ku, Sapporo, Hokkaido, Japan

Received: November 14, 2015; Accepted: December 18, 2015; Published: December 20, 2015


A 31-year-old man was diagnosed with multiple liver abscesses. Despite 4 weeks of antibiotic treatment, his fever persisted with associated pancytopenia and increased liver enzymes. A bone marrow biopsy with aspiration revealed hemophagocytosis, and the diagnosis of Bacteria-Associated Hemophagocytic Syndrome (BAHS) was made. Administration of corticosteroids and Intravenous Immunoglobulin (IVIG) resulted in significant improvement of symptoms. BAHS accounts for 9% of Hemophagocytic Lymphohistiocytosis (HLH) and has a poor prognosis. It can be a diagnostic challenge because it can mimic or coexist with sepsis. Along with proper antibiotic treatment, early co-therapy with immunosuppressive agents may be life saving.

Keywords: Liver abscess; Bacteria-associated hemophagocytic syndrome; Hemophagocytic lymphohistiocytosis; Sepsis


Hemophagocytic Lymphohistiocytosis (HLH) is a lifethreatening disease characterized by extreme inflammation due to an uncontrolled and ineffective immune response [1]. It is caused by a dysregulation in natural killer T-cell function, resulting in activation and proliferation of lymphocytes or histiocytes with uncontrolled hemophagocytosis and cytokine overproduction [2]. HLH can be either primary, with a genetic etiology or secondary, associated with malignancies, autoimmune diseases, or infections. HLH can occur at any age; however, most of published studies are focused on pediatric patients. There are no precise data regarding the incidence of HLH in adults. An estimate of HLH in children <18 years old across ethnicities and races is approximately 1 in 100,000 per year [3]. In a nationwide survey in Japan that included both adult and pediatric patients, the annual incidence of HLH was estimated to be 1 in 800,000 per year [4]. In adults, most of HLH is secondary. HLH related to infections accounts for 40% of the cases, of which 9% is Bacteria-Associated Hemophagocytic Syndrome (BAHS) [5]. A standard treatment of BAHS, however, has not been established. We describe a case of a patient with BAHS induced by liver abscesses. Corticosteroids and Intravenous Immunoglobulin (IVIG) proved to be useful in treating this patient.

Case Presentation

A 31-year-old Japanese man presented to the hospital with oneday history of fever and fatigue. A Computed Tomography (CT) scan revealed multiple liver abscesses (Figure 1). The specific etiology of these abscesses remained elusive. He was treated with broad-spectrum antibiotics with evidence of abscess improvement on imaging; however, daily fever persisted. After 4 weeks of hospitalization, he also developed pancytopenia and increased liver enzymes, at which point he was transferred to our institution.