哈里斯血小板综合征中的血小板和红细胞指数。

Platelet and red blood cell indices in Harris platelet syndrome.

机构信息

Department of Internal Medicine, Division of Hematology, Mayo Clinic, College of Medicine, Rochester, MN, USA.

出版信息

Platelets. 2010;21(4):303-6. doi: 10.3109/09537101003615402.

PMID:20201635

Abstract

Inherited thrombocytopenias, including inherited giant platelet disorders (IGPD) or macro thrombocytopenias are relatively rare, but their prevalence is likely underestimated from complexities of diagnosis and a spectrum of subclinical phenotypes. Harris platelet syndrome (HPS) is the most common IGPD reported from the Indian subcontinent. Of note there are an increased number of hemoglobinopathies reported from the geographic location. We analysed red blood cell and platelet indices of blood donors with HPS from the north eastern part of India and compared them with blood indices of blood donors of south India. We found a statistically significant lower platelet count in blood donors with HPS (median, range) 132 (71-267) vs. 252 (160-478) as compared to donors from south India (P < 0.001). Mean platelet volume (MPV) was higher in donors with HPS 13.1, (range 12-21.9 fl) as compared to donors from south India 7.35 (range 6-9.2 fl) (P < 0.001). This study showed that blood donors with HPS had a low median platelet bio-mass 0.17 (0.10-0.38%) vs. 0.19 (0.13-0.28%) in donors from south India. The platelet distribution width (PDW) was 17.4 (14.9-19.6) in donors with HPS vs. 16.38 (15.2-18.5) in south Indian blood donors (P < 0.001). Thirty-three donors with HPS had a normal platelet count with MPV more than 12 fL. Only donors with HPS had giant platelets and thrombocytopenia on peripheral blood smear examination. None of these donors had Dohle body inclusion in their leukocytes. Compared to donors from south India, donors with HPS had a significantly lower hemoglobin 13.8 (12-16.3 gm/dL) vs. 14.8 (12-18) respectively (P < 0.001) while red distribution width (RDW) was higher in HPS 13.6 (11.5-16.7) vs. 12.8 (11.4-15.1). However we did not find any statistically significant difference in MCV, MCH, MCHC between the two groups. Peripheral blood smear did not show any obvious abnormal red blood cell morphology. In the blood donors with HPS we found a statistically higher MPV, RDW and a lower platelet count and platelet biomass. A population-based study will be helpful in determining the existence of any hemoglobinopathies among subjects with HPS.

摘要

遗传性血小板减少症，包括遗传性巨血小板疾病（IGPD）或巨血小板减少症，相对较为罕见，但由于诊断的复杂性和亚临床表型的多样性，其患病率可能被低估。哈里斯血小板综合征（HPS）是报告来自印度次大陆的最常见的 IGPD。值得注意的是，该地区报告的血红蛋白病数量有所增加。我们分析了来自印度东北部的 HPS 献血者的红细胞和血小板指数，并将其与来自印度南部的献血者的血液指数进行了比较。我们发现 HPS 献血者的血小板计数明显较低（中位数，范围）132（71-267）与来自印度南部的献血者相比为 252（160-478）（P<0.001）。HPS 献血者的平均血小板体积（MPV）较高，为 13.1（范围 12-21.9 fl），而来自印度南部的献血者为 7.35（范围 6-9.2 fl）（P<0.001）。这项研究表明，HPS 献血者的血小板生物量中位数较低，为 0.17（0.10-0.38%），而来自印度南部的献血者为 0.19（0.13-0.28%）。HPS 献血者的血小板分布宽度（PDW）为 17.4（14.9-19.6），而来自印度南部的献血者为 16.38（15.2-18.5）（P<0.001）。33 名 HPS 献血者的血小板计数正常，但 MPV 超过 12 fL。只有 HPS 献血者在外周血涂片检查中出现巨血小板和血小板减少症。这些献血者的白细胞中均未发现 Dohle 小体包涵物。与来自印度南部的献血者相比，HPS 献血者的血红蛋白明显较低，分别为 13.8（12-16.3 gm/dL）和 14.8（12-18）（P<0.001），而 HPS 的红细胞分布宽度（RDW）较高，分别为 13.6（11.5-16.7）和 12.8（11.4-15.1）。然而，我们没有发现两组之间 MCV、MCH 和 MCHC 有任何统计学差异。外周血涂片未显示任何明显的异常红细胞形态。在 HPS 献血者中，我们发现 MPV、RDW 统计学上较高，血小板计数和血小板生物量较低。一项基于人群的研究将有助于确定 HPS 患者中是否存在任何血红蛋白病。