Cramer E, Auclair C, Hakim J, Feliu E, Boucherot J, Troube H, Bernard J F, Bergogne E, Boivin P
Blood. 1977 Jul;50(1):93-106.
The functional capacities of granulocytes in patients with chronic granulocytic leukemia are still a subject of controversy, probably due to the heterogeneity of the abnormalities observed from patient to patient. For a better definition of these abnormalities, 14 patients with untreated chronic granulocytic leukemia were studied. The patients were divided into three groups on the basis of the functional activities of their phagocytosing granulocytes. In four patients (group I), the granulocytes were normal in respect to particle ingestion, nitroblue tetrazolium (NBT)-stimulated reduction, cyanide-insensitive oxygen (O2) consumption, superoxide anion (O2-)-stimulated production, hydrogen peroxide (H2O2) production, and iodination. They also had a normal myeloperoxidase (MPO) content. In four patients (group III), the granulocytes were significantly defective in all of these activities. In the six remaining patients (group II), all the initial metabolic steps of the phagocytosing granulocytes (ingestion, NBT reduction, O2 consumption, O2-production, H2O2 production) were normal, as were the MPO content of the granulocytes, while iodination was strikingly decreased. These metabolic features suggested a degranulation defect which was observed ultrastructurally in the only patient studied among these six. The phagocytosing granulocytes of this patient did not degranulate and no deposits of MPO activity were seen in the phagosomes.
慢性粒细胞白血病患者粒细胞的功能能力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。为了更好地定义这些异常，对14例未经治疗的慢性粒细胞白血病患者进行了研究。根据吞噬粒细胞的功能活性，将患者分为三组。在4例患者（I组）中，粒细胞在颗粒摄取、硝基蓝四唑（NBT）刺激还原、氰化物不敏感氧（O2）消耗、超氧阴离子（O2-）刺激产生、过氧化氢（H2O2）产生和碘化方面均正常。他们的髓过氧化物酶（MPO）含量也正常。在4例患者（III组）中，粒细胞在所有这些活动中均存在明显缺陷。在其余6例患者（II组）中，吞噬粒细胞的所有初始代谢步骤（摄取、NBT还原、O2消耗、O2产生、H2O2产生）均正常，粒细胞的MPO含量也正常，而碘化显著降低。这些代谢特征提示存在脱颗粒缺陷，在这6例患者中唯一研究的患者的超微结构中观察到了这一缺陷。该患者的吞噬粒细胞未脱颗粒，吞噬体中未见MPO活性沉积。
