The Absolute Erythrocytoses

The initial investigation and classification of the absolute ery-throcytoses is given in Figure 9-3. In the primary erythrocy-toses it is erythropoiesis itself that is defective, whereas in secondary erythrocytoses intrinsically normal erythropoiesis is increased as a response to physiologically or pathologically increased erythropoietin secretion. The only acquired primary type is the clonal myeloproliferative disorder PV, whereas the only congenital primary type described so far is a mutation of the Epo receptor, which may be due to familial or spontaneous mutation (see pathogenesis section). The causes of congenital and acquired secondary erythrocytoses are shown in Table 9-2. The term idiopathic erythrocytosis is used for the heterogeneous group of patients who cannot, at initial investigation, be defined as either primary or secondary erythrocytosis. Either of these diagnoses may become apparent with time, however.

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