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Harrison's Principles of Internal Medicine, 18e
| Part 7. Oncology and Hematology > Section 2. Hematopoietic Disorders > |
Chapter 108. Polycythemia Vera and Other Myeloproliferative Diseases
Sections: Polycythemia Vera and Other Myeloproliferative Diseases: Introduction, Polycythemia Vera, Primary Myelofibrosis, Essential Thrombocytosis, Further Readings.
Topics Discussed: pathophysiology of blood disorders; pathophysiology of neoplasia.
Excerpt:"The World Health Organization (WHO) classification of the chronic myeloproliferative diseases (MPDs) includes eight disorders, some of which are rare or poorly characterized (Table 108–1) but all of which share an origin in a multipotent hematopoietic progenitor cell; overproduction of one or more of the formed elements of the blood without significant dysplasia; a predilection to extramedullary hematopoiesis, myelofibrosis; and transformation at varying rates to acute leukemia. Within this broad classification, however, significant phenotypic heterogeneity exists. Some diseases such as chronic myelogenous leukemia (CML), chronic neutrophilic leukemia (CNL), and chronic eosinophilic leukemia (CEL) express primarily a myeloid phenotype, while in others such as polycythemia vera (PV), primary myelofibrosis (PMF), and essential thrombocytosis (ET), erythroid or megakaryocytic hyperplasia predominates. The latter three disorders, in contrast to the former three, also appear capable of transforming into each other...."
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