目标:在潜在的疾病修饰剂如Janus激酶抑制剂的时代,骨髓(BM)纤维化的准确分级和分化与评估疾病分期和治疗效果越来越相关。然而,过去使用了不同的纤维化分级模型,没有一致性,包括世界卫生组织的建议。目前的评分系统仅基于网状蛋白纤维化。因此,对胶原和骨硬化程度的额外评估似乎对于区分复杂BM纤维基质的所有成分至关重要。
结果:我们在总共352个样本上评估了有关染色技术和网状蛋白纤维化解释的问题和陷阱。此外,我们建议对目前的胶原沉积和骨硬化的分级和单独评分进行小的修改。在盲法评估中,在11名血液病理学家中测试了分级的可重复性。总的来说,所有三种评分系统的评分者间可靠性介于0.898和0.926之间。
结论:BM纤维化的标准化评估与网织蛋白,建议使用胶原和骨硬化来评估治疗后可能脱钩的纤维基质的各种成分。在这方面,染色质量和实验室标准的应用使一个高度可重复的评分。
OBJECTIVE: In the era of potentially disease-modifying agents such as Janus kinase inhibitors, accurate grading and differentiation of bone marrow (BM) fibrosis has become more relevant to assess staging of disease and therapeutic effects. However, different fibrosis grading models have been used in the past without uniformity, including the proposal by the World Health Organization. Current scoring systems are based only on
reticulin fibrosis. Therefore, additional assessment of collagen and the grade of osteosclerosis appear to be essential to discriminate all components of the complex BM fibrous matrix.
RESULTS: We evaluated problems and pitfalls regarding staining techniques and the interpretation of
reticulin fibrosis on a total of 352 samples. Furthermore, we propose a minor modification of the current grading and separate scoring for collagen deposition and osteosclerosis. Reproducibility of gradings was tested among 11 haematopathologists in a blinded assessment. Overall, the inter-rater reliability of all three grading systems ranged between 0.898 and 0.926.
CONCLUSIONS: A standardized assessment of BM fibrosis with differentiation between
reticulin, collagen and osteosclerosis is recommended to evaluate the various components of the fibrous matrix which may be delinked after therapy. In this regard, quality of staining and application of laboratory standards enable a highly reproducible scoring.