Unusual, non-anaphylactic transfusion reactions associated with severe IgA and IgM deficiencies and anti-IgA

Abstract

Abstract A few individuals with undetectable IgA develop anti-IgA, which is theorized to promote anaphylactic or anaphylactoid reactions to blood products. Here, we describe a recurrent, unusual reaction in a patient with no IgA or IgM, and anti-IgA present. The patient is a 49-year-old male with cirrhosis secondary to hepatitis C, who presented to the emergency department in January of 2014 with abdominal distension, leg swelling and dyspnea. He was severely anemic with a hemoglobin of 4.5 g/dL. Several minutes into a transfusion, he complained of chills, dyspnea and back pain. The transfusion was discontinued after <10mL, and his symptoms resolved rapidly without further intervention. The patient’s blood pressure, heart rate and temperature before and after the reaction were 134/63 mmHg and 180/64 mmHg, 98 beats/minute (bpm) and 102 bpm, and 98.6⁰ F and 98.7⁰ F, respectively. A laboratory workup for hemolysis was negative pre- and post-transfusion and an “OK to transfuse” order was given if future blood products were indicated. The following day, another transfusion of PRBCs was stopped after <75 mL due to similar symptoms, which again resolved rapidly; no vital signs changes or signs of hemolysis were noted. Because he was receiving other fluids concurrently with the PRBCs, it was recommended to transfuse future products slowly and to premedicate with furosemide. Although he tolerated three subsequent PRBC transfusions during the same admission, he developed the same clinical picture as soon as <75mL of a platelet unit was started. At that time, unbeknownst to Transfusion Medicine, the primary care team ordered immunoglobulin levels and anti-IgA, and the results of these tests were entered into his electronic medical record (EMR) after discharge. Approximately six years later, the patient was readmitted following a fall and was found to be thrombocytopenic. After receiving <30 mL of platelets, he developed back pain and headache and the transfusion was aborted. His wife informed the primary provider that her husband was IgA-deficient. When Transfusion Medicine was notified, a review of his EMR showed undetectable IgA (<6 mg/dL: reference: 46-236 mg/dL) and IgM (<25 mg/dL; reference 43-279 mg/dL) and mildly increased IgG (1787 mg/dL; reference 650-1643 mg/dL) from 2014. Additionally, a high-titer IgG anti-IgA (>1000 U/mL; reference <99 U/mL), had been reported. In lieu of these findings, we changed his transfusion requirements to issue only washed PRBCs and IgA-deficient platelets and plasma, but he has not required any blood products since the last reaction. While a definitive cause and effect has not been established, this case suggests that severe IgA- and IgM-deficiency with IgG anti-IgA may be associated with nonspecific symptoms even with the transfusion of small volumes (<75 mL). To our knowledge, similar reactions have not been previously reported.