Hyperhemolysis syndrome in a patient without a hemoglobinopathy, unresponsive to treatment with eculizumab.

BACKGROUND: Hyperhemolysis is a serious transfusion reaction, most often described in patients with hemoglobinopathies. Hyperhemolysis is characterized by the destruction of host red blood cells (RBCs), in addition to donor RBCs, via an unknown mechanism. STUDY DESIGN AND METHODS: We present the case of a 58-year-old woman with treated human immunodeficiency virus and a normal hemoglobin (Hb) electrophoresis who developed hyperhemolysis in the setting of a delayed hemolytic transfusion reaction (DHTR). RESULTS: The patient was ABO group B and had a previously identified anti-Fy(b) alloantibody. After transfusion of Fy(b)--RBCs, she developed a DHTR and was found to have anti-E, anti-C(w), anti-s, and an additional antibody to an unrecognized high-frequency RBC alloantigen. Subsequent transfusion of ABO-compatible RBCs that were negative for Fy(b), E, C(w), 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. Severe hemolytic reactions also occurred despite pretransfusion conditioning with eculizumab. The Hct and clinical symptoms slowly improved after the cessation of transfusions and treatment with erythropoietin and steroids. This case demonstrates several noteworthy features including hyperhemolysis in a patient without a Hb disorder, the development of an antibody to an unknown RBC antigen, and the failure of eculizumab to prevent intravascular hemolysis after transfusion. CONCLUSION: Hyperhemolysis is not restricted to patients with hemoglobinopathies. Whether eculizumab offers any benefit in the hyperhemolysis syndrome or in the prevention of intravascular hemolysis due to RBC alloantibodies remains uncertain.

How to find, recruit and maintain rare blood donors.

PURPOSE OF REVIEW: Since the early 1960s, it was recognized that patients with very complex serology may be limited in the availability of rare blood for transfusion. Over the years, there have been publications about the quest to meet those needs. Although the world's literature on how to find, recruit and maintain rare blood donors is not overwhelming, there are quite a few pearls. This review will seek out those pearls published in 2007-2009 and provides some insight from a perspective of having a responsibility for a nation of patients requiring rare blood for over 15 years. RECENT FINDINGS: Most pertinent publications have focused on a particular country and the data gathered by a particular regional area or the national rare donor program. It is clear that the definition of 'rare' varies from country to country. A blood type rare in one country may not be considered rare in another. A few of the publications that will be reviewed are specific to donor recruitment or specific details regarding a particular blood type. Recently, with the advent of semi-automated equipment to assist in DNA analysis, there has been a volley of articles on the use of this equipment.Without effective rare donor programs, there is a risk that transfusion needs may not be met. Hemovigilance concentrates on adverse events related to blood transfusions, and the event that happens when rare blood is not available may be that the patient dies without the transfusion they need. SUMMARY: The need for rare blood has been recognized for nearly 50 years, and there are some very effective programs across the world, but not all the areas of the world are equally supplied. The International Society of Blood Transfusion Working Party for Rare Donors is a vital link in the worldwide goal of providing rare blood to the patients who need it.