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Kidney Week

Abstract: PO0122

Severe AKI Associated to Acquired Autoimmune Hemolytic Anemia and Hemophagocytic Syndrome: A Case Report

Session Information

Category: Trainee Case Report

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Santos, Afonso, Nephrology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Lisboa, Portugal
  • Lima, Anna, Nephrology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Lisboa, Portugal
  • Gaspar, Ana, Nephrology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Lisboa, Portugal
  • Brás, Ana Catarina, Nephrology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Lisboa, Portugal
  • Carrilho, Patricia S., Nephrology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Lisboa, Portugal
  • Soto, Karina, Nephrology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Lisboa, Portugal
Introduction

Autoimmune hemolytic anemia (AIHA) is a rare entity with an annual incidence of 1-3/100.000.Secondary AIHA is associated to lymphoproliferative disorders (LD), autoimmune diseases, drugs, and less frequently to infections, being an unusual cause of AKI.Hemophagocytic lymphohistiocytosis (HLH) is an uncommon syndrome of excessive immune activation that can be triggered by infection that disrupts immune homeostasis.Herein, we present a very rare case of a patient with AKI requiring RRT associated to severe AIHA and HLH.

Case Description

A 46-year-old caucasian man was admitted with fever, anuria and severe anemia.Three days prior to admission he had vomiting, diarrhoea and oliguria. Physical examination revealed normal BP, fever and pale skin.Blood tests showed SCr 8.8mg/dL (baseline 0.9mg/dL), metabolic acidosis and urine dipstick Hb3+.He had AIHA with Hb 6.7g/dL, LDH 1194U/L; haptoglobin<30mg/dL; total bilirubin 1.35mg/dL; and positive Coombs test.Blood smear revealed atypical lymphocytes and rare blasts; platelet count was normal.HLH was diagnosed based on clinical (fever, splenomegaly) and laboratorial criteria, with high ferritin (46833ng/mL); triglycerides 308mg/dL, high levels of IL2 receptor (13547pg/mL). Sedimentation rate was 85mm/h and CRP 19mg/dL.LD were ruled out through bone marrow biopsy, lymphocyte immunophenotyping test and full-body CT scan.Viral serology revealed EBV infection.
Early treatment was initiated with corticosteroids associated to IV immunoglobulin (IVIG).Kidney recovery started after 10d and haematological recovery started after 3d of IVIG with remission after 15d.The patient remains in complete kidney and hematological remission at 6mo FU.

Discussion

Secondary HLH occurs in response to an inciting stimulus and has been associated to malignancy and infection, particularly with EBV.AIHA is a condition characterized by autoantibodies directed against erythrocytes and can be secondary to EBV infection.Intravascular hemolysis can lead to AKI due to erythrocyte destruction and release of free Hb causing tubular injury by heme proteins’ toxicity.This case illustrates the severe presentation of a rare complex entity of AIHA associated to HLH, both caused by EBV infection, and complicated with severe AKI.Early diagnosis was challenging and determined the good outcome.