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骨髓增殖性疾病。分类及诊断特征,特别着重于慢性粒细胞白血病和原发性骨髓化生。

Myeloproliferative disorders. Classification and diagnostic features with special emphasis on chronic myelogenous leukemia and agnogenic myeloid metaplasia.

作者信息

Hyun B H, Gulati G L, Ashton J K

机构信息

Thomas Jefferson University Hospital, Philadelphia, Pennsylvania.

出版信息

Clin Lab Med. 1990 Dec;10(4):825-38.

PMID:2272176
Abstract

Leukocytosis, mild anemia, thrombocytosis, and panhyperplasia in the marrow characterize the early stages of most of the CMPD, whereas extramedullary hematopoiesis (such as in the spleen or liver), peripheral cytopenias (anemia, leukopenia, or thrombocytopenia), and myelofibrosis, with or without osteosclerosis, reflect the changes seen in the later stages. Transitions among the different CMPD and termination in acute leukemia or marrow failure also are common. CML often is characterized by leukocytosis and the presence of the entire spectrum of granulocytes (mature and immature) in the blood and marrow, reduced LAP, hypercellularity with prominent granulocytic hyperplasia in the marrow, Ph chromosome, and bcr-abl gene rearrangement. Typical features of AMM include leukoerythroblastosis, teardrop poikilocytosis, anemia, increased or normal LAP, prominent megakaryocytic hyperplasia in the marrow, dyshematopoiesis, and hyperplastic or fibrotic/sclerotic marrow.

摘要

大多数慢性髓性增殖性疾病(CMPD)的早期阶段特征为白细胞增多、轻度贫血、血小板增多以及骨髓全血细胞增生,而髓外造血(如在脾脏或肝脏)、外周血细胞减少(贫血、白细胞减少或血小板减少)以及骨髓纤维化(伴或不伴骨硬化)则反映了后期所见的变化。不同CMPD之间的转变以及以急性白血病或骨髓衰竭告终也很常见。慢性粒细胞白血病(CML)通常的特征是白细胞增多,血液和骨髓中存在全谱系粒细胞(成熟和未成熟),白细胞碱性磷酸酶(LAP)降低,骨髓细胞增多伴显著的粒细胞增生,费城染色体(Ph染色体)以及bcr-abl基因重排。非典型慢性髓性白血病(AMM)的典型特征包括幼粒-幼红细胞血象、泪滴状异形红细胞症、贫血、LAP增加或正常、骨髓中显著的巨核细胞增生、造血异常以及增生性或纤维化/硬化性骨髓。

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