Marquetty C, Labro-Bryskier M T, Perianin A, Hakim J
Am J Hematol. 1984 Apr;16(3):243-54. doi: 10.1002/ajh.2830160306.
Functional and enzyme activity of neutrophils in patients with agnogenic osteomyelofibrosis (AOM) is still a subject of controversy, probably owing to the heterogeneity of the abnormalities observed from patient to patient, and the absence of longitudinal studies performed during the course of the disease. For a better definition of these abnormalities, 40 patients with untreated AOM were studied from the time of diagnosis, and in 12 cases, neutrophil function and enzyme activity were reassessed at one-year intervals for 3-5 years. The results showed that all abnormalities were serum-independent. Mean ingestion rate in neutrophils from patients were similar to that of the controls but individual values were more varied, with some very high and some low ingestion rates. Histochemical latex ingestion and nitroblue tetrazolium reduction were lower in patients than in controls, as were cyanide-insensitive O2 consumption and superoxide anion and H2O2 production. Mean iodination was also decreased and, in most patients, was related to a decrease in H2O2 production. However, in eight patients H2O2 production was normal, although an iodination deficiency was found. Myeloperoxidase activity was low in five of these cases. In the other three patients this discrepancy was not explained. The longitudinal study of 12 patients showed that defects observed in the early stages of the disease remained or were aggravated, and that new abnormalities appeared during the course of the disease.
原因不明的骨髓纤维化（AOM）患者中性粒细胞的功能和酶活性仍是一个有争议的话题，这可能是由于患者之间观察到的异常存在异质性，以及在疾病过程中缺乏纵向研究。为了更好地定义这些异常，对40例未经治疗的AOM患者从诊断时开始进行研究，其中12例患者在3至5年的时间里每隔一年重新评估中性粒细胞功能和酶活性。结果表明，所有异常均与血清无关。患者中性粒细胞的平均吞噬率与对照组相似，但个体值差异更大，有些吞噬率非常高，有些则很低。患者的组织化学乳胶吞噬和硝基蓝四氮唑还原低于对照组，对氰化物不敏感的耗氧量、超氧阴离子和过氧化氢生成也低于对照组。平均碘化也降低，并且在大多数患者中，与过氧化氢生成减少有关。然而，在8例患者中，尽管发现碘化缺乏，但过氧化氢生成正常。其中5例患者的髓过氧化物酶活性较低。在另外3例患者中，这种差异无法解释。对12例患者的纵向研究表明，在疾病早期观察到的缺陷持续存在或加重，并且在疾病过程中出现了新的异常。
