Pujol-Moix N, Hernández A, Escolar G, Español I, Martínez-Brotóns F, Mateo J
Departament d'Hematologia. Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Spain.
Haematologica. 2000 Jun;85(6):619-26.
Exact diagnosis is sometimes difficult in patients presenting with a slight bleeding diathesis, prolonged bleeding times, non-specific aggregometric abnormalities, and/or mild thrombocytopenia. The objective of this study was to evaluate the use of platelet ultrastructural morphometry in detecting a partial d-storage pool disease in such patients.
Platelets from 52 patients and 15 controls were fixed immediately in glutaraldehyde in White's saline without anticoagulant and processed for transmission electron microscopy. Using computer-assisted morphometry, the size and shape of the platelets were measured, as were the size and number per platelet of the dense- and a-granules. Ultrastructural morphology of the above and other intraplatelet structures was observed.
Twenty-four cases were diagnosed as having a partial d-storage pool disease. Mean platelet area (2.28 microm(2)) and maximum diameter (2.58 microm) were significantly greater in patients than in control subjects (1.64 microm(2) and 2. 25 microm, respectively) but discoid shape was preserved. Mean dense-granule number was decreased, both per platelet and per microm(2) of platelet area (patients 0.22 and 0.09; controls 0.42 and 0.24). Seven patients also had a marked decrease in a-granules, resulting in a significantly lower mean number of granules per microm(2 )(patients 2.43; controls 3.15). Additionally, the patients' platelets had significant increases in both lipid droplets and surface-connected canalicular system.
A partial dense-granule deficiency, sometimes associated with partial a-granule deficiency, should be borne in mind faced with patients who have a slight bleeding diathesis, non-specific platelet dysfunction tests and/or mild thrombocytopenia of unknown origin. Platelet ultrastructural morphometry is useful in diagnosing this condition.
对于存在轻微出血倾向、出血时间延长、非特异性凝集异常和/或轻度血小板减少的患者，有时难以做出准确诊断。本研究的目的是评估血小板超微结构形态测定法在检测此类患者的部分d-贮存池病中的应用。
52例患者和15例对照者的血小板立即在不含抗凝剂的怀特氏盐水中用戊二醛固定，并进行透射电子显微镜检查。使用计算机辅助形态测定法，测量血小板的大小和形状，以及致密颗粒和α颗粒的大小和每个血小板中的数量。观察上述及其他血小板内结构的超微结构形态。
24例被诊断为部分d-贮存池病。患者的平均血小板面积（2.28μm²）和最大直径（2.58μm）显著大于对照者（分别为1.64μm²和2.25μm），但仍保持盘状形态。平均致密颗粒数量减少，无论是每个血小板还是每平方微米血小板面积中的数量（患者分别为0.22和0.09；对照者为0.42和0.24）。7例患者的α颗粒也明显减少，导致每平方微米颗粒的平均数量显著降低（患者为2.43；对照者为3.15）。此外，患者的血小板中脂滴和表面连接小管系统均显著增加。
面对有轻微出血倾向、非特异性血小板功能测试和/或不明原因的轻度血小板减少的患者，应考虑存在部分致密颗粒缺乏，有时还伴有部分α颗粒缺乏。血小板超微结构形态测定法有助于诊断这种情况。
