2020 Volume 57 Issue 3 Pages 314-317
IgA and haptoglobin (HP) deficiencies are rare diseases. Their first sign in patients is often anaphylaxis when they receive blood transfusions. Here, we describe the case of a 2-year-old Japanese girl with neuroblastoma who showed anaphylactic shock. She was referred to us for the treatment of neuroblastoma. As a result of receiving intensive chemotherapy, she presented with pancytopenia. Immediately after the second transfusion, she developed dyspnea, hypotension, and bradycardia, and stopped breathing. She was diagnosed as having anaphylactic shock and rescued by cardiopulmonary resuscitation. Subsequently, we used washed PC and RBC. As a result, there was no recurrence of the anaphylaxis. Serological tests showed that Hp was absent and anti-Hp antibodies were detected. In addition, molecular analyses demonstrated congenital Hp deficiency in this patient. The anaphylactic shock was induced by the marked interaction between the transfused Hp and the anti-Hp antibodies. The relationship between congenital Hp deficiency and neuroblastoma warrants investigation.
