Paroxysmal nocturnal haemoglobinuria in pregnancy

Paroxysmal nocturnal Haemoglobinuria PNH is an acquired clonal pluripotent hematopoietic stem cell disorder resulting due to a mutation of the phosphatidylinositol glycan A PIG A gene A variety of symptoms are observed in the PNH patients including anemia thrombosis and cytopenias nbsp The disease is markedly contradicting with chronic symptoms thereby requiring supportive therapy including folic acid and iron replacement periodical transfusions and glucocorticoids and anticoagulation therapy A new treatment strategy including inhibition of the terminal complement cascade with a monoclonal antibody eculizumab has also been discussed nbsp The researches in the past reveal that antibody eculizumab tends to decrease the complement mediated intravascular hemolytic and need for periodical transfusion It also enhances the quality of life in patients with this rare disorder nbsp Although there rare chances of incidence of PNH during pregnancy it can lead to major maternofetal complications Although only a few studies have been done on pregnant patients with PNH these findings depict significant breakthroughs in guiding safe pregnancy and lowering the amount of risk involved earlier