Delayed Hemolytic Transfusion Reaction (DHTR) uknown RBC antibody

Status: 
Ready to upload
Record number: 
1211
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Gupta: Case Report
Time to detection: 
3 days
Alerting signals, symptoms, evidence of occurrence: 
Three days after the transfusion of 3 units of Fyb - RBCs her Hct declined to 33.9% from a value of 25.9% in the previous day. The patient developed a DHTR and was found to have anti-E, anti-Cw, anti-s, and an additional antibody to an unrecognized high-frequency RBC alloantigen. Subsequent transfusion of ABO-compatible RBCs that were negative for Fyb, E, Cw , and s antigens resulted in immediate intravascular hemolysis. In the absence of bleeding, her hematocrit (Hct) decreased to 10.2%. An extensive serologic evaluation failed to identify the specificity of the high-frequency antibody.
Demonstration of imputability or root cause: 
The clinical symptoms confirmed on day 14 after transfusion as well as the multiple biological and serological signs were compatible with a DHTR. On days 3 through 12 the Hct continued to decline despite repeated transfusions of cross-match-compatible blood. DAT was found to be positive 2+ (IgG) on day 10 after transfusion, and an eluate demonstrated an anti-s as well as a pannaglutinin. Likely, anti-Fyb, anti-s, anti-E, anti-Cw and an alloantibody to a high prevalence antigen were identified in the patient’s plasma.The diagnosis of a hyperhemolytic syndrome triggered by the DHTR was established in accordance to the failure of the Hct to increase despite the transfusion of antigen-negative, cross-match-compatible RBCs, a low reticulocyte response, markedly elevated LDH, absent haptoglobin, and gross hemoglobinuria.
Imputability grade: 
3 Definite/Certain/Proven
Groups audience: 
Suggest new keywords: 
DHTR
Hyperhemolitic syndrome
red blood cell transfusion
eculizumab
Reference attachment: 
Suggest references: 
Gupta, S., Fenves, A., Nance, S.T., Sykes, D.B. and Dzik, W.S. (2014). Hyperhemolysis syndrome in a patient without a hemoglobinopathy, unresponsive to treatment with eculizumab. Transfusion 55(3):623-628. DOI: 10.1111/trf.12876.
Expert comments for publication: 
The article presents the case of a 58-year-old woman with treated human immunodeficiency virus and a normal hemoglobin (Hb) electrophoresis who developed hyperhemolisis in the setting of a delayed hemolytic transfusion reaction (DHTR). Hyperhemolysis syndrome is a rare but potentially catastrophic condition characterized by hemolysis of both transfused and autologous RBCs. This case demonstrates a number of notable features: 1) It confirms that hyperhemolisis is not restricted to patients with hemoglobinopathies; 2) it occurred in the context of a DHTR that involved an alloantibody to an unknown high frequency antigen that could not be resolved despite extensive reference laboratory investigation; and 3) it demonstrates the failure of eculizumab therapy to block acute intravascular lysis occurring at the time of transfusion. Patient recovered by withholding further transfusions while receiving corticosteroid and eritropoietina.