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Kidney Week

Abstract: SA-PO1167

From Tick Bite to Cytokine Storm: Ehrlichiosis-Triggered Hemophagocytic Lymphohistiocytosis in a Kidney Transplant Recipient

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Bohling, Amy Marie, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Azam, Muhammad Jibran, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Johnson, Jeshanah, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Shawar, Saed, Vanderbilt University Medical Center, Nashville, Tennessee, United States
Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by uncontrolled hyperinflammation in which the immune system targets one's own tissues and organs. Clinical manifestations of this disorder include fever, hepatosplenomegaly, cytopenias, hyperferritinemia, and hypertriglyceridemia. As it can be rapidly fatal, it is important to recognize and treat the cause quickly to prevent organ failure. Etiologies include infections, malignancies, rheumatologic disorders, and immunodeficiencies, and each one dictates a different treatment to prevent the worsening of disease. Here we present a case of a tick-borne illness, Ehrliciosis, as the etiology of HLH.

Case Description

70 y.o. male with history of hypertension, heart failure, end stage renal disease status post deceased donor kidney transplant (10/2020) who presented after syncopal episode, found to be febrile and hypotensive requiring pressors and with an oliguric acute kidney injury. Other notable findings on presentation were elevated liver function tests (LFTs): 179 AST (baseline normal) and 68 ALT (baseline normal), and cytopenias: WBC 2.9, Hgb 11.2 (baseline normal), platelets 33 (baseline normal). Prior to presentation over a course of two weeks, he developed progressive fatigue, dyspnea/orthopnea, productive cough, decreased oral intake, and intermittent fevers/chills, with poor response to outpatient antibiotics. He missed immunosuppressive medications during this time. Ferritin on admission was >33k and uptrended quickly. Soluble il-2 R also came back >8k. HLH was suspected, and a full infectious panel, bone marrow biopsy, and full body imaging was pursued. Infectious workup and imaging were largely unremarkable except for a tick borne study that showed ehrlichia. In the meantime, the patient's creatinine went from 4.2 (baseline 2) to 9.6 over the course of a week requiring multiple episodes of dialysis for uremia. With a week of doxycycline, his ferritin downtrended significantly, and his HLH resolved.

Discussion

This case is a striking reminder of how dramatically HLH can present with fever, pancytopenia, AKI, encephalopathy, uremia, and shock. Fever and cytopenia are not unusual in kidney transplant recipients; this case underscores the importance of keeping HLH and tick-borne illnesses on the differential, especially in the immunosuppressed population.