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23 Myeloproliferative Neoplasms
CHAPTER
KEY TERMS
allogeneic stem cell transplantation dysm egakaryocytopoiesis myeloproliferative neoplasms
BCR-ABL1 fusion gene extram edullary hem atopoiesis polycythem ia vera
chlorom as hypercellular prim ary m yelo brosis
chronic m yelogenous leukem ia (CML) leukem oid reaction progenitor cells
cytochem istry leukocyte alkaline phosphatase (LAP) splenom egaly
cytoreduction minimal residual disease throm bocythem ia
LEARNING OUTCOMES
General characteristics and classi cation ■ Describe the clinical signs and symptoms of PRV.
■ Name the four diseases classi ed as myeloproliferative neoplasms ■ List criteria for establishing a diagnosis of PRV.
(MPNs). Compare the characteristics of PRV and other types of polycythemias.
Differentiate and compare the peripheral blood and bone marrow ■ Explain the factors that in uence prognosis.
characteristics and diagnostic laboratory results for these disorders. ■ Name the control and treatment methods in PRV.
■ Brie y describe the common abnormalities of hemostasis and coag- Primary myelo brosis
ulation in MPNs. State the other name for primary myelo brosis.
■ Report the general prognostic features of MPNs. ■ Brie y describe the epidemiology of primary myelo brosis.
■
■ Criteria that indicate a transformation of an MPN into a blast crisis. ■ Name the predominant clinical manifestation in primary myelo brosis.
■ Brie y explain general treatment approaches to MPNs. Describe the pathophysiology of primary myelo brosis.
Contrast labs for MPNs, MDSs, and acute leukemia. ■ De ne and describe the consequences of dysmegakaryocytopoiesis.
Describe molecular gene mutations in MPS BCR-ABL, p53, JAK2, ■ Brie y characterize the genetic mutation pro le of primary myelo brosis.
and other mutations and expression of growth factor receptors. ■
■ Delineate the clinical signs and symptoms of primary myelo brosis.
Chronic myelogenous leukemia ■ Name the cellular components of a leukoblastic peripheral blood picture.
■ Name the subtypes of chronic myelogenous leukemia (CML). ■ Describe the life span prognosis in primary myelo brosis.
Explain the treatment approach to primary myelo brosis.
■ Describe the epidemiology of CML. ■
Assess the role of platelet-derived growth factor (PDGF) in brosis
■ Explain the pathophysiology of this leukemia. associated with primary myelo brosis.
■ Delineate the usefulness of detection of genetic alterations in CML.
■ Compare the clinical signs and symptoms of this leukemia in the Essential thrombocythemia
three phases of CML. ■ List and describe the major criteria and other ndings for the diag-
■ Describe the cellular aspects of CML. nosis of essential thrombocythemia.
■ Explain the use of leukocyte alkaline phosphatase (LAP) in the diag- ■ Describe the epidemiology of essential thrombocythemia.
nosis of CML compared to a leukemoid reaction. ■ Outline the major features of essential thrombocythemia.
■ Characterize modes of treatment and prognostic features in CML. ■ Explain the most common disorders in patients with essential
Compare the characteristics of atypical chronic myeloid leukemia thrombocythemia.
(aCML) with chronic neutrophilic leukemia (CNL). ■ State the classic laboratory ndings in essential thrombocythemia.
Describe the spectrum of eosinophil disorders ranging from benign ■ Discuss platelet function ndings in essential thrombocythemia.
eosinophilia to eosinophilic leukemia. ■ Compare the bone marrow architecture of essential thrombocythe-
mia with other MPNs.
Polycythemia vera
■ Review the relationship between essential thrombocythemia and PRV.
■ State the other names that might be used to refer to polycythemia ■ Report the treatment approach to essential thrombocythemia.
rubra vera (PRV).
■ Describe the epidemiology of PRV. Chronic neutrophilic leukemia
■ Name the most striking feature of PRV. Compare the characteristics of chronic myeloid leukemia, aCML,
■ Identify unique genetic abnormality in PRV. and CNL.
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