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Chapter 8
Myeloproliferative disorders
Introduction
Definitions and classification
The current World Health Organization (WHO) classification of hematologic malignancies groups the diverse myeloproliferative disorders (MPDs) into 2 general categories: chronic myeloproliferative diseases and myelodysplastic/myeloproliferative diseases (Table 8-1). The latter group includes patients whose conditions exhibit both dysplastic morphology and proliferative features (eg, extramedullary hematopoiesis) at the time of initial presentation. In addition, there are a few exceptionally rare myeloproliferative syndromes that have been clearly described but that are not formally included in the WHO scheme, such as chronic basophilic leukemia or the 8p11 myeloproliferative syndrome.
The 4 major or "classic" Epidemiology
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 Ambiguous cases Laboratory features Course and prognosis Disease progression and leukemic transformation Thrombohemorrhagic risk Therapy Phlebotomy Thromboprophylaxis and symptomatic therapy Acute thrombosis management Cytoreductive therapy Hematopoietic stem cell transplantation Therapy for secondary acute myeloid leukemia in polycythemia vera Pregnancy with PV
Essential thrombocythemia
Pathobiology Clonality JAK2 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 Stem cell transplantation
Chronic idiopathic myelofibrosis (agnogenic myeloid metaplasia)
Pathobiology Marrow microenvironment, CD34+ cells, and clonality Cytogenetic and molecular findings Clinical features Differential diagnosis Laboratory features Course and prognosis Therapy General considerations Splenectomy Newer agents Stem cell transplantation
Hypereosinophilic syndromes, including chronic eosinophilic leukemia
Introduction Clinical features Course, prognosis, and therapy
Chronic myeloid leukemia
Pathobiology Philadelphia chromosome and BCR-ABL Downstream effects of BCR-ABL Disease progression Clinical features in chronic phase Laboratory features of chronic phase Differential diagnosis Distinction from other causes of neutrophilia Molecular assays for BCR-ABL detection Ph-negative, BCR-ABL-negative CML and chronic neutrophilic leukemia Course, prognosis, and features of advanced disease Chronic phase Accelerated phase Blast phase (leukemic progression) Therapy Imatinib mesylate and other treatments aimed at achieving remission Stem cell transplantation Autologous transplantation Allogeneic transplantation Related versus unrelated donors Transplant-related complications Graft-versus-leukemia effect and reduced-intensity conditioning regimens Posttransplant relapse General treatment approaches Palliative therapeutic approaches Therapy for advanced disease Imatinib and cytoreductive therapies Allogeneic transplantation Imatinib resistance Future approaches
Mastocytosis
Introduction Diagnosis Therapy   CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
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