Myeloproliferative neoplasms (MPNs) are a phenotypically diverse group of stem cell—derived clonal disorders characterized by proliferation of 1 or more of the components of the myeloid lineage (ie, erythroid, granulocytic, megakaryocytic, or mast cell). In 1951, William Dameshek first used the term myeloproliferative disorders (MPDs) and grouped together these 4 similar and overlapping clinicopathologic entities (ie, chronic myelogenous leukemia [CML], primary myelofibrosis [PMF], polycythemia vera [PV], and essential thrombocythemia [ET]). In 2008, the World Health Organization (WHO) revised the classification of MPDs and renamed this group of disorders as MPNs to underscore their clonal nature (Table 14-1). The . . . [Full Text of this Article]


Epidemiology
 

Chronic myelogenous leukemia, BCR-ABL1 positive
 
Epidemiology
Pathobiology
Clinical features
Diagnostic criteria
Treatment
Imatinib mesylate and other treatments aimed at achieving remission
Second-generation TK inhibitors
Stem cell transplantation
Allogeneic transplantation
Graft-versus-leukemia effect and reduced-intensity conditioning regimens
Therapy for advanced disease
Imatinib and cytoreductive therapies
Allogeneic transplantation
Course and prognosis
Chronic phase
Accelerated phase
Blast phase (leukemic progression)

Chronic neutrophilic leukemia
 
Epidemiology
Pathobiology
Clinical features
Diagnostic criteria
Treatment
Course and prognosis

Systemic mastocytosis
 
Epidemiology
Pathobiology
Clinical features
Diagnostic criteria
Treatment
Course and prognosis

Myeloid (and lymphoid) neoplasms associated with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1
 
Myeloid and lymphoid neoplasms associated with PDGFRA rearrangement
Epidemiology
Pathobiology
Clinical features
Diagnostic criteria
Treatment
Course and prognosis
Myeloid neoplasms associated with PDGFRB rearrangement
Epidemiology
Clinical features
Diagnostic criteria
Treatment
Course and prognosis
Myeloid and lymphoid neoplasms associated with FGFR1 abnormalities
Epidemiology
Pathobiology
Clinical features
Diagnostic criteria
Treatment
Course and prognosis

Chronic eosinophilic leukemia, not otherwise specified
 
Epidemiology
Clinical features
Diagnostic criteria
Treatment
Course and prognosis

Polycythemia vera
 
Pathobiology
Clonality
JAK2 mutations
Other biologic abnormalities
Clinical features
Differential diagnosis
Absolute polycythemia versus relative polycythemia
Primary erythrocytosis versus secondary erythrocytosis
Familial polycythemic states
Diagnostic criteria and molecular testing
Ambiguous cases
Laboratory and histopathologic features
Course and prognosis
Disease progression and leukemic transformation
Thrombohemorrhagic risk
Therapy
Phlebotomy
Thromboprophylaxis and symptomatic therapy
Acute thrombosis management
Cytoreductive therapy
Hematopoietic SCT
Therapy for secondary AML in PV
Pregnancy with PV

Essential thrombocythemia
 
Pathobiology
Clonality
JAK2/MPL mutations
Other biologic features
Clinical features
Differential diagnosis and laboratory features
Distinction from reactive thrombocytosis or other myeloid disorders
Laboratory features
Bone marrow and cytogenetic findings
Course and prognosis
Disease progression
Thrombohemorrhagic risk
Therapy
General considerations
Cytoreductive therapy
Pregnancy
Prevention and management of thrombosis and hemorrhage
SCT or transformation to acute leukemia

Primary myelofibrosis
 
Pathobiology
Marrow microenvironment, CD34+ cells, and clonality
Cytogenetic and molecular findings
Clinical features
Diagnosis
Laboratory features
Clinical, morphologic, and histopathologic features
Course and prognosis
Therapy
General considerations
Anemia
Splenomegaly
Newer agents
Stem cell transplantation

Myeloproliferative neoplasm, unclassifiable
 
Epidemiology
Clinical features
Course and prognosis