Critical Care: Student/Resident Case Report Poster - Critical Care I |

A Case of Progressive Multifocal Leukoencephalopathy in a Patient With EBV-Associated Hemophagocytic Lymphohistiocytosis FREE TO VIEW

Andree Koop, MD; Ali Zaied, MD; Jose Yataco, MD
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Mayo Clinic Florida, Jacksonville, FL

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

Chest. 2016;150(4_S):374A. doi:10.1016/j.chest.2016.08.387
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care I

SESSION TYPE: Student/Resident Case Report Poster

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

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially life-threatening hyperinflammatory syndrome. Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating infection of the central nervous system (CNS). We describe the clinical course of a patient diagnosed with secondary HLH and PML.

CASE PRESENTATION: A 61-year-old male with a history of seizures was transferred from another hospital for obscure gastrointestinal (GI) bleeding. Three weeks earlier, he presented with left sided weakness and epigastric pain. Evaluation revealed persistent fevers, hepatosplenomegaly, pancytopenia, a right frontal lobe lesion and a ferritin level of 43,000 ng/mL. A bone marrow biopsy (BMBx) was concerning for HLH. He was treated with steroids and etoposide for HLH and broad spectrum antibiotics for neutropenic fever. Hospital course was complicated by recurrent hematochezia requiring frequent blood product transfusions and he was transferred to our facility for further evaluation. Upon arrival, he was sedated and mechanically ventilated. Neurological exam revealed expressive aphasia and left sided weakness. Small bowel enteroscopy showed a jejunal stricture with diffuse ulceration and bleeding. Biopsy showed necrosis and inflammation without evidence of malignancy. Soluble IL-2 receptor was 6280 pg/mL. EBV serum PCR was 11,800 copies/mL. The working diagnosis was EBV associated HLH. We repeated BMBx and reviewed the outside BMBx; both showed increased histiocytes but no hemophagocytosis. MRI of the brain showed a demyelinating frontal lobe lesion. Cerebrospinal fluid PCR for JC virus was negative. Brain biopsy confirmed PML with positive tissue staining for papovavirus and in-situ hybridization for JC virus. The family decided to pursue hospice care and the patient passed away one week after discharge.

DISCUSSION: HLH is a disease of immune dysregulation characterized by excessive cytokine production leading to multi-organ system damage. EBV is the most common infectious cause. The patient fulfilled diagnostic criteria for HLH. 1 PML is a severe CNS opportunistic infection caused by JC virus in immunocompromised individuals. 2 To our knowledge, this is the first reported case of the co-occurrence of PML and HLH. The exact association is unclear. It is possible that the immunosuppressive state associated with HLH led to the reactivation of JC virus and PML. Both diseases have poor a prognosis and high mortality rates.

CONCLUSIONS: This is a case report of the co-occurrence of HLH and PML. Both diseases are associated with immunosuppression and have a poor prognosis.

Reference #1: Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, et al. Adult haemophagocytic syndrome. Lancet 2014; 383:1503-1516

Reference #2: Carruthers RL, Berger J. Progressive multifocal leukoencephalopathy and JC Virus-related disease in modern neurology practice. Multiple sclerosis and related disorders 2014; 3:419-430

DISCLOSURE: The following authors have nothing to disclose: Andree Koop, Ali Zaied, Jose Yataco

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