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Adult-Onset Hemophagocytic Lymphohistiocytosis: A Rare Cause of Fevers and Multiorgan Failure in a Young Healthy Woman FREE TO VIEW

Ryan Dunn*, MD; Maggie Davis Hovda, MD
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University of Chicago, Chicago, IL

Chest. 2012;142(4_MeetingAbstracts):295A. doi:10.1378/chest.1385046
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SESSION TYPE: Critical Care Student/Resident Cases

PRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PM

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease marked by overactive histiocytes and lymphocytes and is associated with a high mortality(1). We present a case of HLH in an otherwise healthy young adult female who presented with recurrent fevers and multi-organ failure.

CASE PRESENTATION: A 20 year old previously healthy female presented to a community hospital with fevers to 105 F, body aches, and generalized weakness. The patient had a leukocytosis to 19.3 and a mild transaminitis. Chest radiography, blood, CSF and urine cultures were negative. Serologies and PCR studies for mycoplasma, syphilis, CMV, EBV, HSV, HIV and acetominophen level were negative. The patient was given broad spectrum antibiotics. She had persistent fevers and was transferred after one week to our institution. Multiple laboratory and imaging studies did not reveal an occult infection. The patient developed a worsening transaminitis with AST/ALT peaking at 8766/1115, a coagulopathy (INR of 1.9) and a lactic acidosis of 12.9. She was anemic at 6.5 and thrombocytopenic at 63. An abdominal MRI revealed splenomegaly. The patient developed cardiorespiratory failure requiring intubation and vasopressors. Ferritin was elevated at 28,000. The patient underwent a bone marrow biopsy which revealed hemophagocytosis. She was started on dexamethasone and etoposide. Over two weeks the patient’s liver failure and hypotension resolved and she was extubated.

DISCUSSION: Secondary HLH may be associated with various infectious, inflammatory and malignant triggers(2). HLH is marked by inappropriate overstimulation of cytotoxic lymphocytes that lead to macrophage activation and high levels of cytokines(1,2). Characteristic features include fever, hepatosplenomegaly, CNS disease, cytopenias, transaminitis, hypofibrinogemia, high tryglyceride levels, hemophagoctyosis in bone marrow or CSF fluid, and elevated markers of T-cell activity(3). Treatment requires immunosuppresion with steroids, etoposide and CSA per the HLH 94 protocol with frequently HSCT(1,2). With current treatment 5-year survival is approximately 50%(2). This case displayed many characteristic features of HLH but given the relative rarity of the disease other infectious syndromes were considered first and the diagnosis delayed.

CONCLUSIONS: Hemophagocytic lymphohistiocytosis is a rare disorder that is marked by immune dysregulation that can lead to multi-organ failure and death. Early diagnosis is essential.

1) Fischer A. Primary Immune Deficiency Diseases. In: Kasper et al. Harrison’s Principles of Internal Medicine. 18th ed New York, NY: MCGraw-Hill 2011:2704

2) Trottestam H et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood 2011;118(17):4577-84

3) Henter JI et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48(2):124-31

DISCLOSURE: The following authors have nothing to disclose: Ryan Dunn, Maggie Davis Hovda

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University of Chicago, Chicago, IL




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