Kidney Week

Abstract: SA-PO1005

The Ticking Time Bomb: A Case of Hemophagocytic Lymphohistiocytosis Triggered by Ehrlichiosis in a Kidney Transplant Recipient

Session Information

• Transplantation: Clinical - 4
  October 26, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Transplantation

• 2102 Transplantation: Clinical

Authors

• Naing, Aung Sitt, Eastern Virginia Medical School, Norfolk, Virginia, United States

Aung Sitt Naing, MD

• Bademian, Sean, Eastern Virginia Medical School, Norfolk, Virginia, United States

Sean Bademian, MD

• Rogers, Ralph, Sentara Medical Group, Norfolk, Virginia, United States

Ralph Rogers, MD

• Rust, Harlan C., Nephrology Associates of Tidewater LTD, Norfolk, Virginia, United States

Harlan C. Rust, MD

• McCune, Thomas R., Nephrology Associates of Tidewater LTD, Norfolk, Virginia, United States

Thomas R. McCune, MD, FASN

Introduction

Human Monocytic Ehrlichiosis (HME) is rare in kidney transplant recipients despite immunosuppression and risk of direct transmission from the donor kidney. Disease severity ranges from mild symptoms to life-threatening illness in immunocompromised patients. There are cases of Hemophagocytic Lymphohistiocytosis (HLH) triggered by HME. However, there have been no such reports in adult kidney transplant recipients.

Case Description

A 60-year-old female with a history of living donor kidney transplant 3 years prior presented with a few days of altered mental status and fever. Initial labs showed pancytopenia, elevated liver enzymes, acute kidney injury. Her hospital course was marked by worsening encephalopathy and pancytopenia requiring serial platelet and RBC transfusions. Empirical antibiotic therapy was initiated for meningitis. CSF analysis was unremarkable. Her condition rapidly deteriorated into profound shock requiring intubation and CRRT. She was started on dexamethasone 10 mg Q12 hours for suspected HLH due to ferritin of 44,000, persistent coagulopathy, pancytopenia, hypertriglyceridemia, liver injury and sCD25 of 14856. Despite no known tick bites, further workup was pursued for tickborne disease due to known association of HLH and HME which revealed a positive E. chaffeensis DNA PCR. A 21-day course of doxycycline was initiated and other anti-infectives discontinued. She improved drastically with resolution of pancytopenia, coagulopathy and shock. She was extubated and taken off CRRT.

Discussion

The incidence of HME has risen significantly from 2000-2019 in Southeastern regions of US. While serology tests in an acute phase has poor sensitivity, PCR testing can be diagnostic. HLH is an exceedingly rare but life-threatening condition caused by dysregulated activity of immune system characterized by consumptive coagulopathy and shock. While HME is effectively treated with doxycycline, treating secondary HLH includes moderate-dose dexamethasone and addressing the underlying cause. Etoposide, IVIG and plasma exchange have been used in severe cases. Mortality ranges from 20-88%. Diagnosis of HME and HLH remains very challenging for clinicians. HME-induced HLH can be considered in fever, pancytopenia, coagulopathy and worsening shock when there is no clear cause identified in kidney transplant recipients living in endemic areas.