G.A. Dudina
Loginov Moscow Clinical Scientific Center of the Moscow Health Department, Moscow
Dudina G.A. ― Cand. Med. Sc., Senior Researcher of the Department of Oncohematology and Secondary Immunodeficiency Diseases of the Loginov Moscow Clinical Scientific Center of the Moscow Health Department
86 Entuziastov highway, Moscow, Russian Federation, 111123, tel. +7-916-650-85-77, e-mail: dudina_gal@mail.ru
Abstract. Paroxysmal nocturnal hemoglobinuria is an acquired, clonal, geneticdisease of blood cells, which characterized absence of CD55 and CD59 inhibitors of the complement system on the surface cells. Absence of the inhibitors is a reason ofintravascular hemolysis which caused thrombosis: the most dangerous PNH complication. High PNH risk groups were identified in a guideline of the International PNH interesting group and one of themis patients with myelodysplastic syndrome. There is described a clinical case of significant PNH clone detectioninan elderly patient with MDS and PNH treatment outcomeswith eculizumab.
Key words: paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome, diagnostic, eculizumab.