A Patient with Paroxysmal Nocturnal Hemoglobinuria in whom G-CSF Administration was Remarkably Effective against Recurrent Gastrointestinal Infections and Hemolytic Episodes Caused by Cellular Immunodeficiency

We report an elderly patient with paroxysmal nocturnal hemoglobinuria (PNH), having recurrent gastrointestinal infections and hemolytic episodes caused by cellular immunodeficiency due to a decreased number of T cells. On admission to our hospital, the patient had normal neutrophil count and function, but had decreased T cell count, decreased mitogenic reaction to PHA and ConA, and negative reaction to the tuberculin test. Thus, a cellular immunodeficiency state was suggested and postulated to be due to decreased T cell count caused by the loss of PNH clone of T cells. We previously reported that the administration of G-CSF to normal donors of allogeneic peripheral stem cell transplantation increases the lymphocyte count. Based on this experience, we administered G-CSF to this patient. This resulted in an increase in the T cell count, normalization of the mitogenic response to PHA and ConA, positive reaction to the tuberculin test and increases in the blood levels of Thl cytokines and Th2 cytokines, with an improvement in the gastrointestinal infections and hemolytic episodes. This suggested that G-CSF administration resulted in the recovery of cell-mediated immunity in the gastrointestinal mucosa. At 9 months after the start of G-CSF administration, these complications have not recurred. This is the first report of a patient of this type and suggests that G-CSF administration may be useful in the treatment of elderly PNH patients with cellular immunodeficiency.