Critical Care: Fellow Case Report Poster - Critical Care III |

A Break in the Case: How PET-CT Led to a Diagnosis of Hemophagocytic Lymphohistiocytosis FREE TO VIEW

Benjamin Deaton, MD; Katherina Avilas, MD; Nicholas Villalobos, MD; Michel Boivin, MD
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University of New Mexico, Albuquerque, NM

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):257A. doi:10.1016/j.chest.2016.08.270
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SESSION TITLE: Fellow Case Report Poster - Critical Care III

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Hemophagocytic lymphohystiocytosis (HLH) is an uncommon diagnosis that is difficult to establish as it often resembles other critical illnesses. We present a case in which PET (Positron Emission Tomography) identified lymphoma (an HLH-associated diagnosis) and led to acquiring vital information that confirmed HLH.

CASE PRESENTATION: A 61-year-old woman was transferred to the University of New Mexico Hospital (UNMH) for FUO after a month long admission to an outside hospital for H1N1 influenza, distributive shock, respiratory failure, and pancytopenia. Her pancytopenia had required multiple transfusions and a bone marrow biopsy had been performed. Once at UNMH, an extensive workup still had not revealed an etiology. A PET scan was performed to further evaluate for occult infection or malignancy. Despite a Computerized Tomography (CT)-Chest/Abdomen performed 2 days prior, the PET revealed multiple hypermetabolic lymph nodes and a porta hepatis mass that were not previously detected. The bone marrow biopsy was then obtained from the outside hospital and showed moderate hemophagocytosis. A diagnosis of HLH was confirmed and the patient was started on dexamethasone and etoposide. Fine needle aspiration of the porta hepatis mass showed likely lymphoma.

DISCUSSION: HLH can be suspected based on criteria from HLH-2004 guidelines which include molecular identification of HLH-associated genes or at least 5 of 8 criteria: Fever ≥ 38.5 C, splenomegaly, two peripheral blood cytopenias, hypertriglyceridemia or hypofibrinoginemia, hemophagocytosis in the bone marrow, low NK cell activity, ferritin > 500ng/mL, and sCD25 count ≥ 2400/mL. Initially, our patient met 5 of 8 criteria but they were thought to be non-specific. While the diagnosis of HLH was still entertained, other etiologies for her FUO were also considered. However, the results of the PET were instrumental in the decision to obtain the outside bone marrow slides. While hemophagocytosis was not initially reported, it was identified on secondary review confirming the diagnosis.

CONCLUSIONS: HLH is a rare, yet increasingly-suspected, life-threatening syndrome that remains difficult to diagnose. In this patient with FUO, PET played a critical role in leading to the diagnosis of HLH.

Reference #1: Henter JI, et al. for the Histiocyte Society. HLH-2004: Diagnostic and Therapeutic Guidelines for Hemophagocytic Lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-131.

DISCLOSURE: The following authors have nothing to disclose: Benjamin Deaton, Katherina Avilas, Nicholas Villalobos, Michel Boivin

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