Posttransfusion hyperhemolysis is arrested by targeting macrophage activation with novel use of Tocilizumab.

BACKGROUND: Hyperhemolysis syndrome (HHS) is a posttransfusion complication most frequently seen in sickle cell disease (SCD), characterized by rapid destruction of transfused and autologous red blood cells (RBCs), resulting in reticulocytopenia and a decrease in hemoglobin to below pretransfusion levels. Additional RBC transfusion can be life threatening. Most patients improve with intravenous immune globulin and steroids, but in refractory cases, hyperhemolysis may result in multiorgan failure and death in the absence of salvage therapy. The exact pathophysiology of HHS remains uncertain, yet new insights suggest that RBC destruction is driven by activated macrophages. Therefore, we propose that antimacrophage therapy may represent an effective treatment. CASE REPORT: A case of life-threatening HHS, refractory to intravenous immune globulin and steroids, in a patient with SCD is presented. Marked elevation in ferritin, an indirect marker of macrophage activation, a negative direct antiglobulin test, and the absence of RBC alloantibodies was noted. A hemoglobin nadir of 2.1 g/dL and resultant hypoxemia-induced organ failure prompted the use of tocilizumab, an interleukin-6 receptor monoclonal antibody. Hemoglobin-based oxygen carrier-201, a cell-free polymerized bovine hemoglobin, was used to support the patient during critical anemia. RESULTS: Hemolysis resolved and ferritin dramatically decreased after administration of tocilizumab, which was well tolerated. A full recovery was achieved. CONCLUSION: This case highlights both a novel and successful approach to managing refractory transfusion-induced hyperhemolysis with tocilizumab and provides further evidence supporting the role for macrophage activation in the destruction of RBCs in antibody-negative HHS. We propose that tocilizumab is an effective and rapid salvage therapy for refractory HHS.

How shall we transfuse Hippolyta?

The US Department of Defense recently made the decision to open direct ground combat roles to women. Blood product transfusion is an essential component of the US Military guidelines for tactical combat casualty care and damage control resuscitation, but blood transfusion carries with it the specific side effect of alloimmunization-a uniquely significant side effect for young women who may desire subsequent pregnancies. Presently to be considered are the changes that may need to be made to blood transfusion in the setting of battlefield medicine to optimally care for combat-injured women, as a majority of the existing data regarding the risks of transfusion in the trauma setting involve predominantly men. This article delves into the possibility of a new cohort of women at risk for hemolytic disease of the fetus and newborn, the need for women's health professionals to appropriately counsel women considering serving in direct ground combat roles about this specific risk, and the appropriate steps that should be considered to provide these women optimal medical care.

Seeing is believing: A review of apheresis therapy in the treatment of ophthalmologic disease.

Apheresis procedures have a role in treatment of disparate diseases involving many different organ systems. Often the disease processes where apheresis plays a role in treatment are considered "orphan diseases"-relatively rare disease processes that lack specific pharmaceutical agents or established treatment protocols. Many of these disease processes can affect the eye with devastating results for the eyesight of these patients. The unique ability of apheresis to affect disease by modifying blood plasma and modulating disease-causing agents therein renders apheresis procedures valuable tools in the treatment of certain ophthalmologic diseases. This review comprehensively evaluates the role of apheresis in the treatment of ophthalmologic diseases of the eye and surrounding orbit including age-related macular degeneration, bilateral diffuse uveal melanocytic proliferation, paraneoplastic retinopathy, atopic keratoconjunctivitis, sympathetic ophthalmia, and endocrine-associated ophthalmopathy. Apheresis procedure parameters are provided for the apheresis practitioner based on review of the relevant literature.