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[急性骨髓纤维化(巨核细胞白血病)随访中的β-血小板球蛋白和血小板第4因子]

[Beta-thromboglobulin and platelet factor 4 in a follow-up of acute myelofibrosis (megakaryoblastic leukemia)].

作者信息

Palomera Bernal L, García Díez I

机构信息

Servicio de Hematología y Hemoterapia, Hospital Comarcal Infanta Margarita, Cabra, Córdoba.

出版信息

Med Clin (Barc). 1990 Jun 2;95(1):21-4.

PMID:2146451
Abstract

We report a patient with acute myelofibrosis (AM) in whom a megakaryocytic origin was demonstrated after conventional microscopy, investigation with monoclonal antibodies directed against the glycoprotein complex IIb/IIIa (CD41a) and the platelet peroxidase (PPO) reaction. Thus, a diagnosis of acute megakaryoblastic leukemia (AMGL) was made. It is now known that this megakaryoblastic proliferation is responsible for myelofibrosis as an increased release of platelet-derived growth factor (PDGF), beta-thromboglobulin (BTG), and platelet factor 4 (PF4) develops because ineffective megakaryocytopoiesis and failure of these clonal populations to store the mentioned substances in their alpha granules. At the time of diagnosis, the plasma concentrations of BTG and PF4 were measured and were found to be high. Thus, an increased PDGF level was indirectly assumed, with the subsequent fibroblast stimulation. After treatment with low dose cytosine arabinoside, a clinical, analytical and histological remission was achieved, with a return of BTG and PF4 values to the normal range. It was therefore concluded that the follow up of these parameters is useful for the diagnosis and the establishment of remission criteria in these patients.

摘要

我们报告了一名急性骨髓纤维化(AM)患者,经传统显微镜检查、针对糖蛋白复合物IIb/IIIa(CD41a)的单克隆抗体研究以及血小板过氧化物酶(PPO)反应,证实其起源于巨核细胞。因此,诊断为急性巨核细胞白血病(AMGL)。现已知道,这种巨核细胞增殖是骨髓纤维化的原因,因为无效的巨核细胞生成以及这些克隆群体无法将上述物质储存于其α颗粒中,导致血小板衍生生长因子(PDGF)、β-血小板球蛋白(BTG)和血小板因子4(PF4)释放增加。在诊断时,测量了BTG和PF4的血浆浓度,发现其浓度很高。因此,间接推测PDGF水平升高,随后刺激了成纤维细胞。用小剂量阿糖胞苷治疗后,实现了临床、分析和组织学缓解,BTG和PF4值恢复到正常范围。因此得出结论,对这些参数进行随访有助于这些患者的诊断和缓解标准的制定。

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