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A Rare Case of Hemophagocytic Lymphohistiocytosis FREE TO VIEW

Lawrence Giove, MD; Justin Goralnik, MD; Evan Nadler, MD; Jenna Wald, MD; Prashant Grover, MD
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University of Connecticut, Farmington, CT

Chest. 2014;146(4_MeetingAbstracts):296A. doi:10.1378/chest.1993922
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SESSION TITLE: Critical Care Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Hemophagocytic lymphohistiocytosis is a rare, life-threatening syndrome of excessive immune activation. It occurs most commonly in children and is rare in adults. Prompt initiation of treatment for HLH is essential for the survival of affected patient.

CASE PRESENTATION: A 39 year old female, previously healthy, presented to the hospital with respiratory distress and hypotension. In the ED, she was intubated and started on vasopressor medications. On arrival to the ICU, she was noted to be febrile, minimally responsive, with anasarca and mottled extremities. Initial blood work revealed pancytopenia, hepatitis, renal failure, and a ferritin level greater than 10,000 ng/ml. She was initiated on broad-spectrum antibiotics for sepsis with concerns for toxic shock syndrome. Despite aggressive intervention, she persisted to have high fevers, progressing cytopenias, and rising ferritin levels. A bone marrow biopsy and aspirate revealed increased numbers of histiocytes with intracytoplasmic hematopoetic elements suggestive of hemophagocytosis. Further workup was negative for CMV, EBV, HIV, Tick borne illness, bacterial infection, and rheumatologic diseases. She was diagnosed with idiopathic HLH and started on etoposide and dexamethasone per the HLH-94 protocol. Over the next several weeks she was continued on chemotherapy and had both a laboratory and clinical response. She was discharged to a rehabilitation facility in stable condition.

DISCUSSION: Hemophagocytic lymphohistiocytosis is a rare syndrome of pathological immune activation which can present with unexplained fever, pancytopenia, multi-organ failure, and death. HLH is divided into primary (familial) and secondary subtypes. Secondary HLH is more common in adults and is associated with predisposing and/or triggering factors. Predisposing factors include primary or acquired immunodeficiency, malignancy, and autoimmune disease. Known triggers include infections and medications. Most common associated infections include, EBV, CMV, human herpes virus 8, and HIV, although other viruses have been reported. Diagnosis is based on molecular findings or fulfillment of diagnostic criteria (see table). The goal of therapy for patients with HLH is to suppress inflammation by destroying immune cells. The HLH-94 protocol consists of eight weeks of induction therapy with etoposide and dexamethasone, with intrathecal therapy for those with CNS involvement.

CONCLUSIONS: HLH is a rare, but important differential diagnosis for a patient presenting with shock. Treatment must be initiated as soon the clinical picture fulfills the diagnostic criteria without delay, as mortality rate is very high.

Reference #1: Henter JI, Samuelsson-Horne A, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002; 100:2367.

Reference #2: Jordan MB, Allen CE, et al. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011 Oct 13;118(15):4041-52

DISCLOSURE: The following authors have nothing to disclose: Lawrence Giove, Justin Goralnik, Evan Nadler, Jenna Wald, Prashant Grover

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