Kidney Week

Abstract: FR-PO746

Hemophagocytic Lymphohistiocytosis After Kidney Transplantation

Session Information

• Post-Transplantation and Case Reports
  November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Transplantation

• 2102 Transplantation: Clinical

Authors

• Frauenfeld, Lina, University of Florida College of Medicine, Gainesville, Florida, United States

Lina Frauenfeld,

• Belal, Amer Ashaab, University of Florida College of Medicine, Gainesville, Florida, United States

Amer Ashaab Belal,

• Ruiz Toledo, Alejandro Javier, University of Florida College of Medicine, Gainesville, Florida, United States

Alejandro Javier Ruiz Toledo,

• Mercogliano, Gianna Marie, University of Florida College of Medicine, Gainesville, Florida, United States

Gianna Marie Mercogliano,

• Lord, Shawna, University of Florida College of Medicine, Gainesville, Florida, United States

Shawna Lord,

• Mehta, Rohan V., University of Florida College of Medicine, Gainesville, Florida, United States

Rohan V. Mehta,

• Vrakas, Georgios, University of Florida College of Medicine, Gainesville, Florida, United States

Georgios Vrakas,

• El-hinnawi, Ashraf, University of Florida College of Medicine, Gainesville, Florida, United States

Ashraf El-hinnawi,

• Santos, Alfonso, University of Florida College of Medicine, Gainesville, Florida, United States

Alfonso Santos,

Introduction

Hemophagocytic Lymphohistiocytosis (HLH) is an immunological disorder resulting in blood cell phagocytosis. In kidney transplant (KT) recipients (KTR), HLH is usually secondary to infectious or neoplastic processes. Typical findings of HLH are fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, coagulopathy-related hypofibrinogenemia, liver dysfunction, and elevated ferritin.
Early diagnosis with appropriate treatment is recommended.

Case Description

An 18-year-old man with ESRD due to Focal Segmental Glomerulosclerosis (FSGS) received a deceased donor kidney transplant (DDKT). Immunosuppression regimens were anti-thymocyte globulin for induction, and tacrolimus, mycophenolate, and prednisone for maintenance. On postoperative day (POD) 4, he developed a high-grade fever and acute kidney dysfunction with increased serum creatinine, proteinuria (507 mg/gm), and edema. Allograft biopsy showed isolated glomerulitis. Pertinent laboratory data included 6.8 103 /uL WBC, 1367/947 iu/L AST/ALT, and 2,329 iu/L LDH. Workup for infection revealed positive serum HSV PCR treated with IV acyclovir. Hemodialysis was initiated on POD 10 for anuria and hyperammonemia. The patient eventually met 5 out of 8 required HLH-1994 diagnostic criteria including high SIL-2R level (3171.5 pg/mL), low NK cell (25 cells/uL), and hyperferritinemia (14,397 ng/mL). Etoposide was administered on POD 17 per the HLH-94 protocol along with cyclosporine and dexamethasone. The patient attained renal recovery around POD 50 after completing 9 doses of Etoposide. His hematologic and serum inflammatory markers have since normalized.

Discussion

HLH is a rare syndrome, with extremely high mortality due to an excessive immune activation triggered by events such as infections and immunosuppression that disrupt immune homeostasis. Rapid diagnosis and prompt treatment post-transplant are key to favorable outcomes. In this case, the most likely cause of secondary HLH was HSV infection aggravated by immunosuppressive medications. Current therapy protocols are almost exclusively validated in pediatric patients and for primary HLH. Our case demonstrates the safe and successful application of this treatment protocol in a young adult patient presenting with HLH after a KT.