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A patient with paroxysmal nocturnal haemoglobinuria in whom granulocyte colony-stimulating factor administration resulted in improvement of recurrent enterocolitis and its associated haemolytic attacks
Authors:Fujimi Akihito  Matsunaga Takuya  Kogawa Katsuhisa  Ohnuma Takayuki  Takahira Naoki  Abe Tomoyuki  Kitaoka Keisuke  Kogawa Takahiro  Tanaka Ikuta  Morii Kazuhiro  Terui Takeshi  Sakamaki Sumio  Kato Junji  Kura Toshiro  Maeda Takeshi  Niitsu Yoshiro
Affiliation:4th Department of Internal Medicine, Sapporo Medical University, School of Medicine, Japan.
Abstract:We report an elderly patient with paroxysmal nocturnal haemoglobinuria (PNH), having recurrent enterocolitis and haemolytic attacks associated with cellular immunodeficiency. On admission, the patient had normal neutrophil count and function but a decreased T-cell count, decreased mitogenic reactions, and a negative tuberculin test. Granulocyte colony-stimulating factor (G-CSF) was administered, resulting in an increased T-cell count, normalization of T-cell function, increased blood levels of helper T cell (Th)1 and Th2 cytokines and improvement in the enterocolitis and haemolytic attacks. This suggests that G-CSF may be useful in the treatment of elderly PNH patients with cellular immunodeficiency.
Keywords:paroxysmal nocturnal haemoglobinuria (PNH)    granulocyte colony-stimulating factor (G-CSF)    cellular immunodeficiency
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