Carulli G, Minnucci S, Azzara A, Gianfaldoni M L, Angiolini C, Sagripanti A, Ferretti A, Ambrogi F
Unit of Hematology, University of Pisa, Italy.
Hematol Pathol. 1995;9(1):37-47.
Chronic myeloproliferative diseases, such as chronic myeloid leukemia and polycythemia vera, are associated with neutrophil dysfunction. Very little data is available on essential thrombocythemia (ET). In the current study we evaluated 21 patients with ET. All patients were studied at least 16 weeks after any cytostatic therapy and 10 days after any other therapy. Neutrophil functions were investigated as follows: flow cytometric evaluation of whole blood phagocytosis of opsonized FITC-conjugated E. coli; whole blood chemiluminescence after stimulation with opsonized zymosan and evaluation by an automated, computer-assisted luminometer (LB 950, Berthold); and chemiluminescence and superoxide anion generation by purified neutrophils after f-MLP and PMA stimulation. Chemiluminescence and superoxide anion generation after f-MLP stimulation were found to be significantly lower than in normal subjects, whereas values within the normal ranges were registered after PMA stimulation. Phagocytosis-associated chemiluminescence was found to be impaired both by using zymosan opsonized with autologous plasma and zymosan opsonized with normal plasma, despite a normal phagocytic activity. These data show the presence in ET of a complex neutrophil dysfunction that may be related to an impaired signal transduction during both the phagocytic process and f-MLP stimulation.
慢性骨髓增殖性疾病，如慢性粒细胞白血病和真性红细胞增多症，与中性粒细胞功能障碍有关。关于原发性血小板增多症（ET）的数据非常少。在本研究中，我们评估了21例ET患者。所有患者在接受任何细胞抑制治疗后至少16周以及接受任何其他治疗后10天进行研究。中性粒细胞功能的研究如下：流式细胞术评估调理过的异硫氰酸荧光素（FITC）标记大肠杆菌的全血吞噬作用；用调理过的酵母聚糖刺激后的全血化学发光，并通过自动计算机辅助发光计（LB 950，贝托尔德公司）进行评估；以及在f - 甲硫氨酰 - 亮氨酰 - 苯丙氨酸（f - MLP）和佛波酯（PMA）刺激后纯化中性粒细胞的化学发光和超氧阴离子生成。发现f - MLP刺激后的化学发光和超氧阴离子生成显著低于正常受试者，而PMA刺激后的值在正常范围内。尽管吞噬活性正常，但使用自体血浆调理的酵母聚糖和正常血浆调理的酵母聚糖时，吞噬相关化学发光均受损。这些数据表明ET中存在复杂的中性粒细胞功能障碍，这可能与吞噬过程和f - MLP刺激期间信号转导受损有关。
