Saunders E F, Gall G, Freedman M H
Pediatrics. 1979 Oct;64(4):515-9.
Granulopoiesis was studied in 10 children with Shwachman's syndrome (chronic neutropenia and exocrine pancreatic insufficiency). Marrow proliferative activity assessed by determination of mitotic indices and tritiated thymidine uptake into granulocytic cells was normal. Assay of bone marrow granulocyte colony-forming cells (CFU-C) in a methylcellulose tissue culture system demonstrated normal CFU-C numbers in four patients and reduced numbers in five. The granulocyte colonies formed were indistinguishable from normal colonies morphologically. Production of colony-stimulating activity (CSA) from patients' peripheral blood leukocytes appeared normal when tested on control marrow. No serum inhibitors against CFU-C or CSA could be demonstrated using both control and autologous marrow, and co-culture of patients' peripheral blood lymphocytes with control marrow did not inhibit CFU-C growth. We conclude that in Shwachman's syndrome committed granulocytic stem cells are present, and the numbers detected in vitro vary widely as does the clinical neutropenia. The proliferative activity of recognizable granulocytic cells is normal and neither a deficiency of humoral stimulators nor the presence of serum or cellular inhibitors of granulopoiesis can be demonstrated.
对10名患有施瓦茨曼综合征（慢性中性粒细胞减少症和外分泌性胰腺功能不全）的儿童进行了粒细胞生成研究。通过测定有丝分裂指数和粒细胞对氚标记胸腺嘧啶核苷的摄取来评估骨髓增殖活性，结果正常。在甲基纤维素组织培养系统中对骨髓粒细胞集落形成细胞（CFU-C）进行检测，4例患者的CFU-C数量正常，5例减少。所形成的粒细胞集落在形态上与正常集落无法区分。用对照骨髓进行检测时，患者外周血白细胞产生集落刺激活性（CSA）的情况似乎正常。使用对照骨髓和自体骨髓均未发现针对CFU-C或CSA的血清抑制剂，并且患者外周血淋巴细胞与对照骨髓共同培养并未抑制CFU-C生长。我们得出结论，在施瓦茨曼综合征中存在定向粒细胞干细胞，体外检测到的数量差异很大，临床中性粒细胞减少症也是如此。可识别的粒细胞的增殖活性正常，未发现体液刺激因子缺乏，也未证明存在血清或细胞性粒细胞生成抑制剂。
