Autoimmune hemolytic anemia (AIHA) after HSCT

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Record number: 
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
AIHA after HSCT is a rare but well-recognized complication that presents a significant risk for morbidity and a reported mortality of greater than 50%. The incidence of AIHA after HSCT is between 4 and 6% according to the reference (Hess et al. Transfusion, 2018).
Time to detection: 
Based on the reference (Hess et al. Transfusion, 2018), time to detection varies, but is not acute. The three case studies listed in the reference demonstrate an onset of 70, 170, and 304 days after the hematopoietic stem cell transplantation.
Alerting signals, symptoms, evidence of occurrence: 
Strongly positive DAT with warm autoantibodies against IgG. Severe normocytic, hypochromic anemia. Concurrent septic shock and reticulocytopenia. Biochemical evidence of hemolysis can be supported by bilirubin, LDH, and haptoglobin levels.
Demonstration of imputability or root cause: 
Unrelated donor transplants and chronic graft-versus-host disease (GVHD) have been implicated as risk factors, although the etiology underlying this complication remains unclear (Chang TY et al., 2016, Medicine). The development of autoreactive lymphocytes in the context of failed central and peripheral immune tolerance is thought to play a role (Holbro and Passweg, Hematology AmSoc Hematol Educ Program, 2015).
Imputability grade: 
2 Probable
Groups audience: 
Suggest new keywords: 
Anemia, Hemolytic, Autoimmune
Hematopoietic Stem Cell Transplantation
Suggest references: 
Jennifer Hess, Leon Su, et al. Successful treatment of severe refractory autoimmune hemolytic anemia after hematopoietic stem cell transplant with abatacept. TRANSFUSION 2018;58;2122–2127 Chang TY, Jaing TH, Wen YC, et al. Risk factor analysis of autoimmunehemolytic anemia after allogeneic hematopoietic stem cell transplantation in children. Medicine (Baltimore) 2016;95:e5396. Holbro A, Passweg JR. Management of hemolytic anemia following allogeneic stem cell transplantation. Hematology Am Soc Hematol Educ Program 2015;2015:378-84.
This entry is intended to update record #1957 and I apologize for duplication. I think “Update Record” makes sense in my case; however, when I use that the form coming out of that link does not show Status drop-down box for me to save my review as “First review complete”. Let me know how I can upload a review more properly.
Expert comments for publication: 
Hess et al. Transfusion, 2018 is the primary suggested reference and it discusses three cases that had AIHA after an HSCT. The cases are 9-year-old male, 4-year-old female, and 14-year-old male. The onset of AIHA after HSCT varied but later than 70 days after HSCT. Patients used Abatacept and their clinical status ameliorated and might be an evidence for AIHA occuring in the setting of Treg dysfunction.