Objectives

After completing this chapter, the student will be able to:

1. Define the myeloproliferative disorders.

2. List and discuss classification of the myelopro-liferative disorders.

3. Identify the major cell lines involved with the myeloproliferative disorders.

4. Discuss the pathogenesis of the myeloprolifera-tive disorders.

5. Identify and differentiate clinical features and signs associated with the chronic myeloprolifer-ative disorders.

6. Identify and describe the peripheral and bone marrow abnormalities associated with the chronic myeloproliferative disorders.

7. Compare and contrast the clinical and laboratory features of the chronic myeloproliferative disorders.

8. Identify the diagnostic criteria for the chronic myeloproliferative disorders.

9. Discuss the treatment of and prognosis for the chronic myeloproliferative disorders.

10. Discuss the cytogenetic abnormalities associated with the chronic myeloproliferative disorders.

188 Part III • White Cell Disorders

The chronic myeloproliferative disorders (CMPDs) are a group of disorders that are considered clonal malignancies of the hematopoietic stem cell.1 These disorders include chronic myelogenous leukemia (CML), myelofibrosis with myeloid metaplasia (MMM), polycythemia vera (PV), and essential thrombocythemia (ET). Significant changes have evolved in the last decade with regard to terminology of leukemias and associated disorders. The World Health Organization (WHO) in conjunction with the Society for Hematopathology and the European Association of Hematopathology published a new classification for myeloid and lymphoid neoplasms.1,2 The WHO based their classification on morphology, genetic, immunophenotypic, biological, and clinical features. For lymphoid disorders, the WHO classification uses the Revised European-American Lymphoma (REAL) Classification. The myeloid disorders include the criteria of the French-American-British (FAB) classification and the guidelines of the Polycythemia Vera Study Group (PVSG).3,4 The WHO classification of the chronic myeloproliferative diseases recognizes seven entities.2,3 Table 12.1 lists these entities. 1 These disorders are unified but independent. Each disease has overlapping clinical features but different etiologies. The CMPDs are characterized by proliferation of one or more cell lines and are predominantly mature in cell morphology. The bone marrow shows varying degrees of abnormal proliferation of myeloid, erythroid, and megakaryocytic elements. In the peripheral blood, the red blood cell (RBC), white blood cell (WBC), and platelet counts vary, and each disorder is identified by the predominant cell that is present. Table 12.2 summarizes the characteristics of the CMPDs.

Figure 12.1 Interrelationships of the CMPDs.

Table 12.1

WHO Classification of

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