Risitano Antonio M, Selleri Carmine
Hematology, Department of Clinical Medicine and Surgery, Federico II University of Naples , Italy.
Hematology and Hematopoietic Stem Cell Transplant Center, Department of Medicine and Surgery, University of Salerno , Italy.
Transl Med UniSa. 2014 Feb 4;8:1-3. eCollection 2014 Jan.
Clonal non-malignant hematological disorders are a heterogeneous group of diseases that are particularly challenging for hematologists. Indeed, most obvious and frequent hematological diseases include a broad spectrum of malignancies, such as leukemias, lymphomas, myeloma, and other myeloproliferative or lymphoproliferative disorders. In recent years, all these diseases have been categorized by the WHO according to a novel classification of myeloid and lymphoid malignancies, which takes in account the outstanding progress in our understanding of molecular defects underlying hematological malignancies. Regardless of a number of novel technologies, hematologists continue to deal daily with conditions where a clear diagnosis of a malignancy is missing: this is the case of several clonal hematological disorders, which are considered bona fide non-malignant. Some myelodysplastic syndromes, chronic T and NK disorders of granular lymphocytes, myelofibrosis, monoclonal gammopathies, monoclonal B-cel lymphocytosis, mastocytosis and paroxysmal nocturnal hemoglobinuria are paradigmatic examples of how clonal disorders are clearly different from cancers, even if they may share with hematological malignancies similar molecular, genetic, epigenetic and immunological processes. Indeed, it is not entirely clear whether in individual conditions such pathogenic mechanisms may represent initial step(s) of malignant transformation, making a bridge between these clonal non-malignant disorders and typical hematological cancers. Some of these non-malignant disorders imply specific pathogenic mechanisms and/or clinical course, and so they have been definitely established with their own biological and clinical identity. However, the obvious question whether some of these clonal non-malignant hematological diseases form some a kind of disease-continuum with their corresponding malignant counterpart is still to be answered.
克隆性非恶性血液系统疾病是一组异质性疾病，对血液科医生来说极具挑战性。事实上，最常见的血液系统疾病包括广泛的恶性肿瘤，如白血病、淋巴瘤、骨髓瘤以及其他骨髓增殖性或淋巴细胞增殖性疾病。近年来，世界卫生组织（WHO）根据髓系和淋巴系恶性肿瘤的新分类对所有这些疾病进行了分类，该分类考虑到了我们对血液系统恶性肿瘤潜在分子缺陷认识的显著进展。尽管有许多新技术，但血液科医生每天仍要应对许多无法明确诊断为恶性肿瘤的情况：一些克隆性血液系统疾病就是如此，它们被认为是真正的非恶性疾病。一些骨髓增生异常综合征、慢性颗粒淋巴细胞T和NK细胞疾病、骨髓纤维化、单克隆丙种球蛋白病、单克隆B细胞淋巴细胞增多症、肥大细胞增多症和阵发性夜间血红蛋白尿，都是克隆性疾病与癌症明显不同的典型例子，即使它们可能与血液系统恶性肿瘤共享相似的分子、遗传、表观遗传和免疫过程。事实上，在个别情况下，这些致病机制是否可能代表恶性转化的初始步骤，从而在这些克隆性非恶性疾病和典型血液系统癌症之间架起一座桥梁，目前尚不完全清楚。其中一些非恶性疾病意味着特定的致病机制和/或临床病程，因此它们已凭借自身的生物学和临床特征得以明确界定。然而，这些克隆性非恶性血液系统疾病中的一些是否与相应的恶性疾病形成某种疾病连续体这一明显问题仍有待解答。
