Macro-amylasemia in a patient with selective IgA deficiency and antiphospholipid antibodies

We report an unusual case with macro-amylasemia with coexistent selective IgA deficiency and antiphospholipid antibodies. A 16-year-old girl was referred to us with a history of episodic abdominal pain accompanied by vomiting and diarrhea. Macroamylasemia was demonstrated by precipitation of 99% amylase activity with polyethylene glycol 6000. She had high levels of anticardiolipin IgG and beta2 glycoprotein 1 IgG antibodies in the blood, but no evidence of clinical criteria of antiphospholipid syndrome. In the literature, although macro-amylasemia has been found to occur in a variety of diseases including autoimmune disorders, to our knowledge, this is the first well-documented case of macro-amylasemia associated with selective IgA deficiency and the presence of antiphospholipid antibodies. It is important that clinicians be aware of their existence in order to avoid unnecessary procedures and that the patient is informed of the macro-amylasemia; moreover, it should be stated in the patient's health record.