Treatment of idiophatic neutropenia associated with agammaglobulinemia with human granulocity colony-stimulating factor.

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A 4-year-old boy with neutropenia associated with agammaglobulinemia was treated with recombinant human granulocyte colony-stimulating factor (G-CSF). The patient had a history of pneumocystis carinii pneumonia at 6 months of age and diagnosed as common variable immunodeficiency (IgG; 58 mg/dl, IgA; 41 mg/dl, IgM; 109 mg/dl, IgE<10 IU/ml) associated with neutropenia (<5%). Although he was placed on a regimen of gamma-globulin administration as a monthly intravenous injection, and no episodes of recurrent infections was observed, he was seen at 4 years of age with a perianal abscess. Neutrophil count was ranged less than 5 percent. Chemotaxic and phagocytic function and oxidative product formation of pheripheral neutrophils were conserved. Neutrophilic cells in bone marrow was decreased with a ratio of myeloid to erythroid of 0.75: 1, and levels of metamyelocytes (1%) and band form (4%) and segmented (1%) neutrophils were much decreased. Bone marrow levels of CFU-GM and CFU-E were slightly increased (488/105 cells) and decreased (2.7/5×104 cells), respectively. Peripheral-blood level of neutrophils was increased by 10 fold by provacation test of prednisolone, but not epinephrine.As he was unsuccessfully treated with antibiotics and sulfamethoxazole-trimethoprim, the daily administration of G-CSF (2μg per kg of body weight) was performed for seven days. Treatment with this growth factor has been very well tolerated. There has been no local reaction at the perianal sites, rash and edema. Although no any change was observed in level of peripheral neutrophils and that of bone marrow CFU-GM and CFU-E, percent of neutrophilic cells was much increased (32.8%) with two peaks of myelocytes (10.2%) and band formed cells (11.0%). Taken as a whole, neutropenia of this patient was probably due to maturation disturbance at the level of myelocytes in bone marrow and treatment with G-CSF was efficient for therapy of this disorder.
日本臨床免疫学会の論文

Treatment of idiophatic neutropenia associated with agammaglobulinemia with human granulocity colony-stimulating factor.

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