Abstract: FR-PO745
Hemophagocytic Lymphohistiocytosis in Adult Renal Transplant Patients: A Case Series of Three Patients
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
- Jain, Swati, Montefiore Health System, Bronx, New York, United States
- Pynadath, Cindy T., Montefiore Health System, Bronx, New York, United States
- Ajaimy, Maria, Montefiore Health System, Bronx, New York, United States
- Shi, Yang, Montefiore Health System, Bronx, New York, United States
- Al Azzi, Yorg, Montefiore Health System, Bronx, New York, United States
- Graham, Jay A., Montefiore Health System, Bronx, New York, United States
- Akalin, Enver, Montefiore Health System, Bronx, New York, United States
- Liriano-Ward, Luz E., Montefiore Health System, Bronx, New York, United States
Introduction
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease that presents with non-specific symptoms, leading to a delay in diagnosis and early initiation of treatment.We present 3 cases of HLH in renal transplant patients at our institution.
Case Description
1) 40-year-old female with end stage renal disease (ESRD) on hemodialysis (HD), diabetes mellitus (DM), and hypertension (HTN) who underwent simultaneous kidney-pancreas transplant (SPK) with Thymoglobulin induction on 9/2021, admitted with neutropenia 2 months later. She developed fever, mental status changes, and cholestatic jaundice. CT abdomen showed mild hepatosplenomegaly. Labs showed WBC 0, plt 32, Hgb 6.6, ferritin 11k, CMV 1852, EBV 346, elevated soluble interleukin-2 (sIL-2). Bone marrow biopsy (BMB) revealed hemophagocytic cells. The probability of HLH was 54%-70% based on H score. She was treated with dexamethasone and Etoposide without response. She expired 2 months post presentation.
2) 34-year-old male with chronic kidney disease stage 5, DM, HTN who underwent SPK with thymo induction on 1/2022 admitted with fever and fatigue one month post-transplant. Labs showed WBC 0, plt 27, hgb5.4, ferritin 68k, CMV 1741, EBV 28600, transaminitis, and elevated sIL-2. The probability of HLH was 54-70% based on H score. BMB showed hypocellular marrow (5-10%) with many histiocytes and markedly decreased trilineage hematopoiesis. He received dexamethasone, eltrombopag and cyclosporine without response and expired 6 weeks post presentation.
3) 62-year-old male with DM, HTN, ESRD on HD, who underwent deceased donor kidney transplant with thymo induction on 9/2022, admitted with fever, malaise, and vomiting 4 months post-transplant. Labs showed hgb 6.7, ferritin >100k, elevated triglyceride and sIL-2. CT scan revealed splenomegaly, and BMB showed HLH secondary to histoplasmosis. He received Amphotericin with good response and was discharged 3 weeks after presentation.
Discussion
Idiopathic HLH carries a poor prognosis with high mortality, but HLH secondary to infection has better prognosis if the infection is treated successfully. Early identification and treatment are crucial to optimize outcomes.