Introduction: IgA deficiency is defined as an isolated deficiency of immunoglobulin A,   IgA in serum (< 0.07 g/L) with normal values of IgG and IgM in persons older than 4 years in whom other causes of hypogammaglobulinemia are excluded. Selective IgA deficiency (IgAD) is the most common primary immunodeficiency of humans.  IgAD individuals may be asymptomatic, mildly symptomatic, or suffer from serious respiratory and gastrointestinal infections, autoimmunity, malignancy, and/or allergic conditions. Patients with IgAD may also produce anti-IgA antibodies that are implicated in adverse transfusion reactions.
Method: case repot of kidney transplantation in a patient with complete IgAD.
Results: Female patient, 40 years old, presented with CKD (chronic kidney diseae), after an episode of menometrorrhagia, for which she received a transfusion of 2 doses of erythrocyte concentrate, after which she had an anaphylactic reaction. Transfusion testing of reactions to blood derivatives indicated and confirmed complete IgA immunodeficiency. The patient's father comes from a region where there is Balkan endemic nephropathy, in whom CKD was also confirmed, and father's sister was also on a hemodialysis program. Over the next 5 years, the patient developed terminal renal insufficiency. The patient's mother was considered as a potential kidney donor for transplantation, but she was of incompatible blood group. No IgAD-related diseases, as well as other contraindications for kidney transplantation, were found in the patient in the processing of suitability for treatment by the method of transplantation. The patient also had multiple thrombosis of blood accesses for hemodialysis (AV fistulae). Hematological work-up revealed thrombophilia, presence of mutations in the PAI-1 4/5G gene in homozygous status. After three years spent on dialysis, she was treated with a kidney transplant from a cadaveric donor of compatible blood group and acceptable MM (miss match). In preparation for the transplant, he received boluses of methylprednisolone at a dose of 1 gr/kg body weight, and due to the presence of anti IgA antibodies, plasmapheresis was performed before and after the kidney transplant. The standard immunosuppressive protocol consisted of tacrolimus, MMF and steroid. There were no complications in the peri- and post-transplantation period. There were no episodes of rejection. During follow-up, the patient has stable graft function, without proteinuria. Two cases of kidney transplantation in patients with selective IgAD have been described in the literature.
Conclusion: Patients with selective and complete IgA immunodeficiency and chronic renal insufficiency should be considered as candidates for kidney transplantation with monitoring of anti IgA antibodies and avoidance of transfusions with blood derivatives.