A World Health Organization (WHO) classification of hematopoietic and lymphoid neoplasms has recently been published. This classification was developed through the collaborative efforts of the Society for Hematopathology, the European Association of Hematopathologists, and more than 100 clinical hematologists and scientists who are internationally recognized for their expertise in hematopoietic neoplasms. For the lymphoid neoplasms, this classification provides a refinement of the entities described in the Revised European-American Lymphoma (REAL) Classification-a system that is now used worldwide. To date, however, there has been no published explanation or rationale given for the WHO classification of the myeloid neoplasms. The purpose of this communication is to outline briefly the WHO classification of malignant myeloid diseases, to draw attention to major differences between it and antecedent classification schemes, and to provide the rationale for those differences. (
IntroductionRecently, the World Health Organization (WHO), in conjunction with the Society for Hematopathology and the European Association of Hematopathology, published a new classification for hematopoietic and lymphoid neoplasms. 1 The concepts that underlie this classification were derived from numerous published clinical and scientific studies and from the experience of more than 100 pathologists, clinicians, and scientists from around the world who collaborated to develop this consensus classification. 2 A basic principle of the WHO system is that the classification of hematopoietic and lymphoid neoplasms should utilize not only morphologic findings but also all available information, including genetic, immunophenotypic, biologic, and clinical features to define specific disease entities. Essentially, the WHO classification attempts to incorporate those disease characteristics that have proved to have clinical and biologic relevance into a useful, working nomenclature.For the lymphoid neoplasms, the WHO classification provides refinement of the entities defined in the Revised EuropeanAmerican Lymphoma (REAL) Classification-a system that is now widely used by pathologists and clinicians. 3 The WHO classification of myeloid neoplasms includes many of the criteria of the French-American-British (FAB) Cooperative Group classifications of acute myeloid leukemia (AML) 4 and myelodysplastic syndromes (MDS) 5 as well as guidelines of the Polycythemia Vera Study Group (PVSG) for the chronic myeloproliferative diseases (CMPDs), 6,7 but there are some significant differences. The purpose of this communication is to outline briefly the WHO classification of the myeloid neoplasms, to draw attention to major differences between the WHO and previous classifications of these disorders, and to provide the rationale for these differences. In addition, we wish to address and clarify specific issues concerning the classification that have appeared both prior and subsequent to the publication of the WHO monograph. The WHO classification of the myeloid neop...