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Hemophagocytic Lymphohistiocytosis (HLH) in the Setting of Massive Retroperitoneal Lipomatosis FREE TO VIEW

Pi Chun Cheng, MD; Damien Patel, MD; Gabriel Bosslet, MD
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Indiana University, Indianapolis, IN

Chest. 2014;146(4_MeetingAbstracts):322A. doi:10.1378/chest.1987979
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SESSION TITLE: Critical Care Student/Resident Cases

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 04:30 PM - 05:30 PM

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excess inflammation and immune activation that can lead to progressive multiorgan failure. HLH’s clinical presentation can vary significantly, making its diagnosis challenging in the ICU. We present a case of HLH with massive retroperitoneal lipomatosis, an association which has not yet been described.

CASE PRESENTATION: A 61 year old Caucasian female with rheumatoid arthritis on chronic prednisone presented with severe shock in the setting of febrile neutropenia. Physical exam was significant for generalized anasarca and a cushingoid appearance. Computed tomography of the abdomen revealed large bilateral retroperitoneal masses with significant mass effect on local structures (Image 1). Her shock was unresponsive to aggressive fluids resuscitation, broad-spectrum antibiotics, antifungal therapy, vasopressors, and stress dose steroids. Despite extensive evaluation, no source of infection was identified, and workup for HLH was initiated. She met 5/8 criteria: fever, cytopenia, hypofibrinogenemia, hyperferritinemia, and an elevated soluble CD-25 level (Table 1). HLH treatment with IVIG, dexamethasone, and mycophenolate mofetil was initiated. Her hospital course was complicated by development of multiple organ dysfunction syndrome (MODS) and eventual death on ICU day 15. Subsequent autopsy revealed retroperitoneal masses as benign lipomatosis.

DISCUSSION: HLH is a syndrome of overstimulated immune system, resulting in excess inflammation, massive cytokinemia, diffuse tissue destruction, and multi-organ involvement (1). The presentation of HLH can be variable and nonspecific. Symptoms often overlap with sepsis, which makes diagnosis of HLH difficult, and often delayed in the ICU (1). HLH has been associated with infections, malignancies, autoimmune disorders, and immunodeficiencies (1). Our patient’s only risk factor was her history of rheumatoid arthritis. Prolonged steroid treatment is known to produce lipomatosis, most commonly found in mediastinal, mesenteric, epidural, and retroperitoneal locations (2). In addition, chronic steroid therapy may lead to further risk factors including infections and immunodysfunctions in individuals already susceptible to HLH. To date, this is the first reported case of HLH in the setting of massive retroperitoneal lipomatosis due to chronic prednisone.

CONCLUSIONS: ICU practioners should always keep HLH in their differential diagnosis of refractory shock. We describe a novel association of HLH with prolonged steroid use and massive retroperitoneal lipomatosis.

Reference #1: Raschke R. Hemophagocytic Lymphohistiocytosis: A potentially Underrecognized Association with Systemic Inflammatory Response Syndrome, Severe Sepsis, and Septic Shock in Adults. CHEST. 2011: 933-938

Reference #2: Bouaddi I, et al. Corticosteroid-Induced Multifocal and Extensive Lipomatosis: A Case Report. Open Journal of Pathology, 2011, 1-3.

DISCLOSURE: The following authors have nothing to disclose: Pi Chun Cheng, Damien Patel, Gabriel Bosslet

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