Georgii A, Vykoupil K F, Buhr T, Choritz H, Döhler U, Kaloutsi V, Werner M
Pathologisches Institut, Medizinische Hochschule Hannover, FRG.
Pathol Res Pract. 1990 Feb;186(1):3-27. doi: 10.1016/S0344-0338(11)81008-3.
This Diagnostic Seminar intends to announce that CMPDs can be classified from BMB histologically by a rather simple system, which can be applied by interested histopathologists successfully. The rationale of this classification is to stay within the groups of diseases which are outlined by clinical findings including the peripheral blood and bone marrow smears. The concept of traditional classification as given by the WHO and textbooks, however, has to be revised as follows (1) Primary diseases of CMPDs must be distinguished from advanced disorders. Primary diseases are CML, P. vera, Thrombocythemia, CMGM, and unclassifiable CMPD. (2) Idiopathic, primary myelosclerosis of the bone marrow is a reactive feature consecutive to neoplastic transformation of hematopoiesis, i.e. myeloproliferation. (3) Advanced disorders comprise (3.1.) excess of blasts and blast crisis, and (3.2.) early myelosclerosis, myelosclerosis and myelofibrosis, advanced myelofibrosis. Advanced disorders are designated by a composed term classifying them among the groups of primary disease and specifying the advanced stage by a suffix, so that the underlying disease remains coining the term, even in unclassifiable cases in which only CMPDs can be applied. (4) The CML group must be subtyped into CML of common type versus that with increase or predominance of megakaryocytes. By this system of classification, it seems possible to classify and type the spectrum of variations occurring among CMPDs to a satisfying result.
本次诊断研讨会旨在宣布,慢性骨髓增殖性疾病(CMPDs)可通过一个相当简单的系统在骨髓活检组织学上进行分类,感兴趣的组织病理学家可成功应用该系统。这种分类的基本原理是保持在由临床发现(包括外周血和骨髓涂片)所界定的疾病组内。然而,世界卫生组织(WHO)和教科书给出的传统分类概念必须修订如下:(1)CMPDs的原发性疾病必须与晚期疾病相区分。原发性疾病包括慢性粒细胞白血病(CML)、真性红细胞增多症(P. vera)、血小板增多症、慢性粒单核细胞白血病(CMGM)以及无法分类的CMPD。(2)特发性原发性骨髓纤维化是造血肿瘤转化(即骨髓增殖)后的一种反应性特征。(3)晚期疾病包括(3.1)原始细胞增多和原始细胞危象,以及(3.2)早期骨髓纤维化、骨髓纤维化和骨髓硬化、晚期骨髓纤维化。晚期疾病由一个复合术语指定,该术语将它们归类于原发性疾病组,并通过后缀指定晚期阶段,这样即使在仅能应用CMPDs的无法分类的病例中,基础疾病仍构成该术语。(4)CML组必须细分为普通型CML与巨核细胞增多或占优势的CML。通过这种分类系统,似乎有可能对CMPDs中出现的各种变异进行分类和分型,从而得到令人满意的结果。