Usefulness of BCSH Criteria for Diagnosing Japanese Polycythemia Vera

Abstract

Objectives: The WorldHealth Organization (WHO) classification (2008) is wid ely usedto d iagnose polycythemia vera (PV). However, some patients clinically suspected of PV may not be definitely diagnosed because they do not have a high hemoglobin (Hb) level, one of the main andessential d iagnostic criteria, or because of the lack of or unfilledminor criteria such as bone marrow biopsy, endogenous erythroid colonies, and serum erythropoietin. The British Committee for Standards in Haematology (BCSH) employed hematocrit (Ht), but not Hb, as an indicator in the PV diagnostic guidelines. Furthermore, if the presence of the JAK2 gene mutation is demonstrated, PV can be diagnosed with Ht only by BCSH. Therefore, we evaluated the usefulness of BCSH criteria for diagnosing PV in Japanese patients. Methods: We determinedhematological andclinical differences in 99 patients who met the BCSH criteria (Group A) and69 PV patients who met the WHO criteria (Group B). Results: All patients in Group B also met the BCSH criteria. Thirty patients in Group A (43%) did not fulfill the WHO criteria. No significant differences were observedin white bloodcell andplatelet counts, serum erythropoietin levels, the JAK2V617F allele burd en, or the risk of thrombosis andtransformation into myelofibrosis between Groups A andB. Conclusions: PV cases diagnosedby the BCSH criteria showedthe same clinical picture as those diagnosedby the WHO criteria. In view of actual clinical settings, the diagnostic criteria of BCSH criteria may be useful for the diagnosis of PV in Japanese patients.