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急性巨核细胞白血病

Acute megakaryoblastic leukemia.

作者信息

Gassmann W, Löffler H

机构信息

2nd Department of Internal Medicine, Christian-Albrechts-University of Kiel, Germany.

出版信息

Leuk Lymphoma. 1995;18 Suppl 1:69-73. doi: 10.3109/10428199509075307.

Abstract

In 1985 acute megakaryoblastic leukemia was included in the FAB classification system of hematological neoplasias with the designation of AML M7. It occurs in all age groups with two peaks in distribution. The one is in adults and the other in children 1 to 3 years of age especially in those with Down's syndrome. The diagnosis of AML M7 requires more than 30% of the nucleated bone marrow cells being megakaryoblasts. The more common types of AML MO-M6 have to be excluded by morphological and cytochemical analysis whereas immunology is needed to exclude ALL. The megakaryocytic nature of the leukemia has to be proven by ultrastructural demonstration of platelet peroxidase or by immunological demonstration of CD61, CD42, CD41 on the surface of the leukemic blasts. Megakaryocytic/megakaryoblastic leukemias show a wide morphologic spectrum. In some instance small cells dominate, clearly showing megakaryocytic differentiation with scant amounts of cytoplasm and with nuclei showing dense chromatin. On the other hand, there are cases with larger cells resembling ALL-L2 blasts with moderate amounts of rather basophilic cytoplasm which in some instances contain azurophilic granules. Cytoplasmic blebs and protrusions are the most prominent feature of many cases. The nuclei of these cells are round with more finely reticulated chromatin and with prominent nucleoli. The megakaryoblastic nature of these cells can be suggested by morphology. However, according to our experience there are cases of c-ALL with the very same morphologic picture. Consequently, immunologic phenotyping of these cases is necessary in any instance. Cytochemistry is of limited diagnostic value in megakaryoblastic leukemias. Usually it is used to exclude the more common types of leukemia.

摘要

1985年,急性巨核细胞白血病被纳入血液系统肿瘤的FAB分类体系,命名为AML M7。它在各年龄组均有发生,分布有两个高峰。一个高峰在成人中,另一个在1至3岁的儿童中,尤其是患有唐氏综合征的儿童。AML M7的诊断要求骨髓有核细胞中超过30%为原始巨核细胞。必须通过形态学和细胞化学分析排除更常见的AML MO - M6类型,而排除ALL则需要免疫学检查。白血病的巨核细胞性质必须通过血小板过氧化物酶的超微结构证明或白血病原始细胞表面CD61、CD42、CD41的免疫学证明来证实。巨核细胞/原始巨核细胞白血病表现出广泛的形态学谱。在某些情况下,小细胞占主导,明显显示巨核细胞分化,细胞质稀少,细胞核染色质致密。另一方面,有些病例中的细胞较大,类似于ALL - L2原始细胞,有中等量的嗜碱性细胞质,在某些情况下含有嗜天青颗粒。细胞质小泡和突起是许多病例中最突出的特征。这些细胞的细胞核呈圆形,染色质更细,呈网状,有明显的核仁。这些细胞的原始巨核细胞性质可通过形态学提示。然而,根据我们的经验,有一些c - ALL病例也有相同的形态学表现。因此,在任何情况下,对这些病例进行免疫表型分析都是必要的。细胞化学在原始巨核细胞白血病中的诊断价值有限。通常它用于排除更常见的白血病类型。

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