Hassoun H, Pavlovsky M, Mansoor S, Stopka T
Department of Biomedical Research, St Elizabeth's Medical Center of Boston, Tufts University Medical School, Mass 02135, USA.
South Med J. 2000 Jul;93(7):710-2.
Criteria proposed by the Polycythemia Vera Study Group (PVSG) as well as several derived algorithms are currently used for the diagnosis of polycythemia vera. Although these guidelines have significantly enhanced diagnostic accuracy, they uniformly consider erythrocytosis as the requisite premise for instigating the subsequent workup. We describe the unusual presentation of a patient with microcytic anemia in whom the diagnosis of polycythemia vera was reached using the PVSG criteria and confirmed by in vitro culture assay of erythroid progenitor cells. This case highlights the usefulness of the PVSG criteria, including the red cell mass determination, for the diagnosis of polycythemia vera even in anemic patients. The roles of spleen red cell pooling and plasma volume expansion as major determinants of this unusual presentation are discussed.
真性红细胞增多症研究组（PVSG）提出的标准以及几种衍生算法目前用于真性红细胞增多症的诊断。尽管这些指南显著提高了诊断准确性，但它们均将红细胞增多视为启动后续检查的必要前提。我们描述了一名小细胞贫血患者的不寻常表现，该患者根据PVSG标准被诊断为真性红细胞增多症，并通过红系祖细胞的体外培养测定得到证实。该病例强调了PVSG标准（包括红细胞容量测定）在诊断真性红细胞增多症方面的有用性，即使对于贫血患者也是如此。文中还讨论了脾红细胞蓄积和血浆容量扩张作为这种不寻常表现的主要决定因素所起的作用。
