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Abstract: SA-PO045

AKI Caused by Devastating Hemophagocytic Lymphohistiocytosis Triggered by Multiple Factors

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical‚ Outcomes‚ and Trials

Authors

  • Pascoal, Felipe, Universidade de Sao Paulo Hospital das Clinicas, Sao Paulo, São Paulo, Brazil
  • Vieira Jr., Jose M., Universidade de Sao Paulo Hospital das Clinicas, Sao Paulo, São Paulo, Brazil
  • Andrade, Lucia, Universidade de Sao Paulo Hospital das Clinicas, Sao Paulo, São Paulo, Brazil
Introduction

Hemophagocytic Lymphohistiocytosis (HLH) is an aggressive condition defined by cytokine storm and blood cell phagocytosis. Renal involvement is still poorly characterized, but includes hypoperfusion, tubular damage, nephrotic syndrome and thrombotic microangiopathy. We report a case in which a patient with HLH developed acute kidney injury requiring dialysis leading to death.

Case Description

A 63-year-old with stable HIV and Type 2 Diabetes was referred to the Emergency Department (ER) due to septic shock and an unsolved oral ulcer. For 3 months he had been seen at the Dermatology Clinic because of a slow-growing hard palate lesion, impairing food intake and leading to malnutrition and weight loss. After clinical stabilization at the ER, he was transferred to the ICU, developed significant pancytopenia (Hb 6.7 g/dL, 450 total leucocytes/μL, 41.000 platelets/μL), hyperferritinemia (41.377 mg/dL), hypertriglyceridemia (318 mg/dL) and was diagnosed with Hemophagocytic Lymphohistiocytosis according to HScore and bone marrow aspirate showing hemophagocytosis. Chest Computed Tomography revealed prominent mediastinal lymphadenomegaly, raising suspicion of lymphoma, and PCR for Epstein-Barr Virus was positive both on bone marrow (927.635 uI/mL) and blood (8.903 uI/mL) analyses. Days after guided antibiotic treatment for Klebsiella pneumoniae bloodstream infection, hemodynamic stability, absence of fever, nephrotoxic drug withdrawal and ongoing pancytopenia, serum creatinine levels rose from 1.74 mg/dL to 3.78 mg/dL, BUN levels rose from 67 mg/dL to 105 mg/dL and urinary output decreased from 800 mL/day to 200 mL/day. The patient was placed on Slow Low Efficiency Dialysis due to failure of conservative AKI management. Despite 15 days of treatment with Etoposide and Methylprednisolone, the patient developed painful abdominal distention and deceased from bowel perforation and fecal peritonitis secondary to angioinvasive aspergillosis and candidiasis.

Discussion

HIV patients are predisposed to HLH, which can be triggered by multiple factors, including infection (viral, bacterial and fungal) and malignancy, that likely occurred in this case. Although usually neglected in the pathophysiology of this syndrome, kidney involvement should be acknowledged, emphasized and tackled, as it worsens outcomes translating into high morbidity and mortality rates.