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Abstract: PO1894

TAFRO: A New Cause for Thrombotic Microangiopathy Mimicking Atypical Hemolytic Uremic Syndrome Successfully Treated with Anakinra and Eculizumab

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • O'Brien, Kathryn, UMASS/Baystate, Springfield, Massachusetts, United States
  • Landry, Daniel L., UMASS/Baystate, Springfield, Massachusetts, United States
  • Crisi, Giovanna M., UMASS/Baystate, Springfield, Massachusetts, United States
  • Mulhern, Jeffrey, UMASS/Baystate, Springfield, Massachusetts, United States
  • Braden, Gregory Lee, UMASS/Baystate, Springfield, Massachusetts, United States

Group or Team Name

  • Kidney Care & Transplant Services of New England
Introduction

arTAFRO is syndrome of Castleman's disase with : thrombocytopenia, anasarca, myelofibrosis, AKI & organomegaly. We present a 17 yr old girl with abdominal lymph nodes who rapidly developed anasarca, splenomegaly, AKI requiring dialysis, & respiratory failure requiring mechanical ventilation. After a lymph node bx 2 months later showed multicentric Castelman's, plasma cell variant, we realized she early on had TAFRO.

Case Description

She rapidly developed ansarca, an 18 cm spleen, & abdominal nodes to 1.9 cm. Bacterial cultures, spinal tap, viral resp panel, mono, HIV, HVV-8 levels were normal (NL ). Hg dropped to 6.7 gm/dl without hemolyisis, platelets 57,000 & WBC,15,500. CRP was 32.5 & sed rate 130. Oliguria ensued & creatinine rose to 3.6 mg/dl. Urinalysis had +1 protein, +3 blood with granular casts. ANA, ASO, streptozyme, myeloperoxidae, proetinase -3, serum immunofixation, anti-phospholipid abs, IgG subclasses, anti-mitochondrial & smooth muscle abs, HLA-B27, ADAMSTS-13 levels & abs were NL. IL-2R levels were markedly high at 12,340 pg/ml as were IL-18, 930 pg/ml, CXCL9 107 pg/ml but Il-6 was only 11.3 pg/ml. Bone marrow showed inceased megakaryocytes & no hemophagocytosis. aHUS testing was negative for all geneitc causes & no abs were found to any complements(C) but both, C3, 65 mg/dl & C4, 4 mg/dl were low & the membrane attack complex C5-C9, markedly elevated at > 50 mg/dl. Renal bx showed endothelial swelling & thrombotic micoangioapathy (TM) confirmed on EM with negative IF. 3 pulses of solumedrol daily, dialysis, 2 plasmaphareses before the aHUS panel returned were started along with 2 doses of Anakinra, an anti-IL-1 drug, 4mg/kg sub q separated by 3 days & 1 dose of Eclizumab, 1200 mg iv. She rapidly improved, was extubated & was discharged off dialysis with a NL creatinine & no edema.

Discussion

We conclude: 1). TAFRO can mimic aHUS & present with markedly elevated IL-2R levels rather than IL-6 levels which can fix complement by the classsic pathway leading to high membrane attack complexes adding to capillary leak, anasarca and TM. 2) An Anti IL-1 drug, Anakinra, & a drug inhibiting C5 cleavage to C5a & C5b , Eclizumab can be combined to successfully treat TAFRO 3) TAFRO must now be added to all reviews and textbooks as a new cause for TM & AKI with classic complement pathway activation.