Abstract: TH-PO219
Necrotizing Crescentic Glomerulonephritis after Allogenic Stem Cell Transplant
Session Information
- Fellows/Residents Case Reports: Glomerulonephritis
November 02, 2017 | Location: Hall H, Morial Convention Center
Abstract Time: 10:00 AM - 10:00 AM
Category: Nephrology Education
- 1302 Fellows and Residents Case Reports
Authors
- Anjum, Shehryar, University at Buffalo, Buffalo, New York, United States
- Liu, Lin, University at Buffalo, Buffalo, New York, United States
- Bent-shaw, Luis, SUNY Buffalo, Buffalo, New York, United States
Background
Renal disease in Allogenic stem cell recipients involves glomerular, tubular /vascular injury triggered by chemo/radiation/infection/ischemia/GVHD & Immunosuppression.Rare reports of collapsing FSGS, Fibrillary GN & Anti GBM noted.
Methods
61 y/o female with Myelodysplastic Syndrome treated with Azacitidine & Decitabine ,complicated by refractory thrombocytopenia & anemia .Allogenic stem cell transplant (Brother) June, 2016, complicated by GVHD (Skin, GI and likely Lung). Hospital admissions for seizures due to PRES likely to Tacroliumus use, Influenza A & RSVpneumonia with subsequent pulmonary GVHD. She developed non oliguric AKI .Creatinine 1.1 -> 1.5 . ROS -ve, v/s stable. Physical exam revealed no JVD, Chest with bibasilar crackles.S1,S2 noted, no S3. Abdomen benign, non tender. Lower extremity +2 pitting edema . Urine under light micro. revealed many WBC/ RBCs but no dysmorphic RBC or cast noted. U/S Renal reveals RT. kid12.8 cm & LT. kid 14 cm , minimal increased echogenicity. No hydronephrosis. Subsequent large epistaxis relieved with nasal packing & transfusing platelets. Transferred to ICU for seizures/confusion . With current picture,concerns for TTP/HUS. Plasmapheresis started. Received 3 sessions + steroids with improved mental status.
Cr 1.15 --> 4.5. ADAMST13 in limits .HIT +ve . Urine again revealed no casts however some dysmorphic RBC suspected. Initiated Hemodialysis due to her worsening renal parameters and oliguric state.She underwent Renal Biopsy which revealed 6/6 Glom. with Necrotizing Crescentic pattern, granulomatous interstit.inflammation & necrotizing vasculitis, moderate interstitial fibrosis and tubular atrophy, no glomeruli for IF. EM reveals disrupted ,corrugated Basement membrane .Serologic studies negative except for anti-GBM(179). Started on oral Cyclophosphamide & prednisone,plasmapheresis resumed with no recovery . Treatment subsequently stopped & Steroids tapered off. Per Oncology team started weekly Rituximab 800 mg IV for chronic GVHD. Patient remains dialysis dependent for>8 weeks now deemed ESRD.
Conclusion
Rare case of RPGN with anti-GBM following allogenic stem transplant as a potential complication of GVHD. Interestingly our patient had clinical Pulmonary GVHD. Exposure of collagen type Iv alpha 3 epitope due to immune injury is speculated mechanism however unclear if true relation with Stem cell transplant/GVHD & Anti GBM disease.