Ventura A, Dragovich D, Luxardo P, Zanazzo G
Istituto di Clinica Pediatrica, Policlinico Santa Chiara, Università di Pisa, Italy.
Haematologica. 1995 May-Jun;80(3):227-9.
We report a boy affected by Shwachman syndrome (SS) who presented severe neutropenia and frequent suppurative infections, which we treated successfully with granulocyte colony-stimulating factor (rHuG-CSF). Daily injections of 7.5 micrograms/kg/day significantly increased the absolute neutrophil count and he was free from infections. In order to avoid the risk of side effects and to improve the child's quality of life, we intermittently administered lower doses of rHuG-CSF. A weekly dose of 2 micrograms/kg/day was able to maintain the absolute neutrophil count high enough (0.58-1.2 x 10(9)/L) to prevent suppurative infections. During the follow-up period (2.5 years) we tried suspending rHuG-CSF twice, but the absolute neutrophil count (0.18-0.31 x 10(9)/L) fell significantly and suppurative infections reoccurred (otitis, perianal abscess). rHuG-CSF may be a useful therapeutic agent in patients with symptomatic neutropenia in SS.
我们报告了一名患有施瓦茨曼综合征（SS）的男孩，他出现了严重的中性粒细胞减少症和频繁的化脓性感染，我们用粒细胞集落刺激因子（rHuG-CSF）成功治疗了这些症状。每天以7.5微克/千克/天的剂量注射显著提高了绝对中性粒细胞计数，并且他没有感染。为了避免副作用风险并改善儿童的生活质量，我们间歇性地给予较低剂量的rHuG-CSF。每周以2微克/千克/天的剂量能够将绝对中性粒细胞计数维持在足够高的水平（0.58 - 1.2×10⁹/L）以预防化脓性感染。在随访期（2.5年）内，我们尝试两次暂停使用rHuG-CSF，但绝对中性粒细胞计数（0.18 - 0.31×10⁹/L）显著下降，化脓性感染再次出现（中耳炎、肛周脓肿）。rHuG-CSF可能是治疗SS有症状性中性粒细胞减少症患者的一种有用治疗药物。
