Kidney Week

Abstract: TH-PO0444

Hemophagocytic Lymphohistiocytosis (HLH): What's the Calm Before the Storm?

Session Information

• Hemodialysis: Novel Markers and Case Reports
  November 06, 2025 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Dialysis

• 801 Dialysis: Hemodialysis and Frequent Dialysis

Authors

• Baigam, Nahida, LSU Health New Orleans, New Orleans, Louisiana, United States

Nahida Baigam, MBBS, MD

• Do, Tammy Nguyen, LSU Health New Orleans, New Orleans, Louisiana, United States

Tammy Nguyen Do, MD

• Yazdi, Farshid, LSU Health New Orleans, New Orleans, Louisiana, United States

Farshid Yazdi, MD, MS

Introduction

Hemophagocytic lymphohistiocytosis (HLH) results from life-threatening immune system overactivation, often misdiagnosed due to nonspecific symptoms¹. Urgent immunosuppression is essential to manage hyperinflammation, and emapalumab may be necessary in cases that deteriorate

Case Description

59-year-old male on dialysis presented with abdominal pain, weakness, and shortness of breath, was hypertensive, anemic, had thrombocytopenia. A CT scan revealed liquid stool in the intestines, with no significant thickening of the bowel wall, required NG tube placement and hemodialysis. Next day, he exhibited fever, lethargy, and worsening encephalopathy. Lab results revealed elevated LDH 5410 and a reticulocyte count of 3.2, low haptoglobin <10, and high ferritin levels > 26,000. H-score of 170 suggested a 40-54% probability of HLH, led to initiation of empiric steroids. Infectious workup prompted treatment for C. difficile diarrhea and intubation for worsening sepsis. Later elevated IL-2 receptor levels 3143 were noted. Unfortunately, he deteriorated and suffered cardiac arrest, becoming unresponsive despite resuscitation efforts. Tragically, he passed away before getting Etoposide.

Discussion

HLH is a serious condition with primary and secondary forms, later related to infections, autoimmune disorders, or malignancies¹ Diagnosis requires meeting five out of eight criteria described¹. Presentation mimics infections involves hyperinflammation and rapid deterioration². Ferritin levels above 7,500-10,000 ng/ml greatly enhance specificity². Distinguishing HLH from infectious diseases is crucial because treatment strategies differ³.
Immunosuppressive therapy, especially with concurrent infections, can be risky; thus, the focus should be on managing infections, often starting with corticosteroids and possibly using IVIG^2,3. Etoposide and dexamethasone reduce proinflammatory cytokines, while emapalumab targets interferon-γ for resistant patients^4,5.
C. difficile infections, often linked to antibiotic use, can lead to severe complications⁶. Rarely, HLH patients have developed C. difficile infections, as seen in cases involving EBV-associated T-cell lymphoma and ulcerative colitis with CMV infection^7,8. Both patients tragically lost their lives, like our patient, underscoring the need for vigilant diagnosis, a multidisciplinary approach, and timely initiation of immunosuppression in this context.

Digital Object Identifier (DOI)

• doi: 10.1681/ASN.202525kgyexd