Abstract: TH-PO567
An Atypical Cause of Autoimmune Hemolytic Anemia in a Post Kidney Transplant Patient
Session Information
- Trainee Case Reports - II
October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1802 Transplantation: Clinical
Authors
- Wang, Aileen, Indiana University School of Medicine, Indianapolis, Indiana, United States
- Sharfuddin, Asif A., Indiana University School of Medicine, Indianapolis, Indiana, United States
Introduction
Autoimmune hemolytic anemia (AIHA) is a rare but devastating consequence of solid organ transplantation. Commonly AIHA can be attributed to immunosuppression with calcineurin inhibitors or viral infection. Here we describe a unique cause of AIHA due to Coombs negative IgA associated AIHA that was refractory to both steroid therapy and adjustments in immunosuppression.
Case Description
23-year-old Hispanic female with history of deceased donor kidney transplant in October 2016 (on tacrolimus, sirolimus, prednisone) presented with fatigue, sclera icterus, and jaundice. Recently she was given clindamycin for dental procedure. On lab Hgb low to 4 gm/dL (baseline 12). Work up revealed elevated LDH, low haptoglobin, elevated retic count, and indirect hyperbilirubinemia consistent with hemolytic anemia. Parvovirus B19 was negative. G6PD level was elevated. Peripheral blood smear revealed marked spherocytosis, anisocytosis, and schistocytes. High sensitivity Coombs test was negative. No splenomegaly on abdomen ultrasound. After transfusion Hgb improved to 8.6, and she was discharged on Prednisone 60mg daily. However she was readmitted a week later with Hgb low to 4.8. Cr uptrended to 1.48 mg/dL (baseline 1.2). Tacrolimus was discontinued. PNH flow cytometry and SPEP were unremarkable. Bone marrow biopsy revealed increased erythropoiesis, supportive of peripheral red blood cell destruction. After transfusion Hgb improved to 8. Pt was continued on 60mg Prednisone along with initiation of azathioprine. But one week later, Hgb again low to 4.9. Sirolimus was then stopped. Negative coombs hemolytic panel and a drug induced antibody panel were sent. Negative coombs hemolytic panel detected an IgA autoantibody against red blood cells. Rituximab was initiated (375mg/m2 weekly). One month later, Hgb stabilized in range 13-14 with return of Cr to baseline. Sirolimus was restarted.
Discussion
This case illustrates an extremely rare autoimmune hemolytic process that is Coombs negative, non-drug induced, and steroid refractory in a kidney transplant patient. Splenectomy is usually considered as the first line therapeutic option for steroid refractory IgA associated AIHA, but in a post transplant patient there is the significant increase in infection risk that must be considered. Rituximab therapy though second line was the better choice in this patient who has achieved complete response to therapy.