Neutropenia and monocytopenia in recurrent anaphylactoid reactions after red blood cell transfusions in a woman with immunoglobulin A (IgA) deficiency and anti-IgA

BACKGROUND: Transfusion-associated anaphylaxis has been associated with anti-immunoglobulin A (anti-IgA) of IgG class in patients with IgA deficiency. In recent years, however, the frequency and clinical impact of this syndrome has been questioned. We present a case of recurrent red blood cell (RBC) transfusion-associated anaphylactoid reactions (rigors, hypertension, transient monocytopenia, and neutropenia) associated with anti-IgA. CASE REPORT: An 88-year-old woman developed anemia after a traumatic right humerus fracture. After receiving approximately 100 mL of RBCs she developed rigors and hypertension; her absolute neutrophil and monocyte counts decreased by 73 and 100%, respectively. All symptoms and signs resolved, and her blood counts normalized. A second RBC transfusion was given, with recurrence of rigors, hypertension, neutropenia, and monocytopenia (69 and 100% declines, respectively), along with fever. All tests for hemolysis were negative. She then received two transfusions of washed RBCs, without incident. The patient was found to be deficient in IgA (1000 U/mL). We reviewed case reports, case series, and reviews of IgA deficiency-associated reactions, examining whether hypertensive reactions and acute neutropenia and/or monocytopenia have been associated with anti-IgA reactions. RESULTS: Reports of reactions associated with anti-IgA emphasize hypotension and are generally classified as "anaphylactic or anaphylaxis." No previous reports described neutropenia or monocytopenia. CONCLUSION: Our case suggests that adverse transfusion reactions associated with anti-IgA of IgG class may sometimes be characterized by anaphylactoid features such as rigors and hypertension, with transient monocytopenia and neutropenia.