Immunoglobulin preparations are one of the products of the human plasma fractionation, where the plasma is obtained, in accordance with WHO guidelines from at least 1,000 donors. These preparations contain all IgG subclasses with various antigen characteristics. In clinical practice these drugs are used as replacement therapy in patients with primary and secondary immunodeficiencies as well as immunomodulatory therapy in many autoimmune diseases and systemic inflammatory diseases. Here we present characteristics of i.v. polyvalent, human immunoglobulin preparations available on the Polish market and the possibilities of their use in clinical practice, in children with hematological diseases. Considering the very low consumption of immunoglobulin preparations in our country as compared to other European countries, we would like to draw the attention of medical professionals, especially pediatricians and haematologists, to the benefits that stem from the use of these drugs in the therapy of children with haematological diseases. Our work will also facilitate the choice of an optimal polyvalent human immunoglobulin preparation for a particular patient (Adv Clin Exp Med 2015, 24, 1, 153-159).

Eradication of factor VIII inhibitors using Immune tolerance induction (ITI) treatment is the standard of care for severe haemophilia A patients presenting with factor VIII inhibitors, but is not always effective. A description of the potential immunological tolerance effect of the IgG Fc domain of recombinant factor VIII Fc fusion protein (rFVIIIFc), as well as published experience with rFVIIIFc for ITI in patients with severe haemophilia A and high-titre inhibitors. Review of published literature describing cases of ITI with rFVIIIFc in patients with severe haemophilia A and high-titre inhibitors between November 2015 and June 2018. Four publications has been found. Of 56 patients with haemophilia A who presented with FVIII inhibitors, 28 achieved a negative Bethesda titre (< 0.6) after ITI treatment using rFVIIIFc. Additional patients continued on rFVIIIFc ITI at the time of publication, while a few were reported to have switched to bypass therapy alone or other factors . For those still undergoing ITI, longer follow-up is needed to determine final outcomes. No adverse events were reported. Based on literature review, preliminary evidence of FVIIIFc use in high risk, first-time ITI suggests rapid time to tolerization. For rescue ITI, rFVIIIFc showed therapeutic benefit in some patients who previously failed ITI. These findings give hope but highlight the need for further evaluation in ongoing clinical trials.