Myeloproliferative neoplasm
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Differentiating myeloproliferative neoplasm from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Myeloproliferation, myeloproliferative disorder, myeloproliferative neoplasm
Overview
The MPNs are clonal hematopoietic stem cell disorders characterized by proliferation of one or more myeloid lineages in the bone marrow with relatively normal maturation resulting in increased numbers of granulocytes, red blood cells and platelets in the peripheral blood. This is in contrast to the ineffective erythropoiesis usually observed in the myelodysplastic syndromes.Sequestration of excess blood cells in the spleen or liver, extramedullary hematopoiesis and leukemic cells infiltration, these events either alone or in combination were found to cause spleenomegaly and hepatomegaly.
MPNs can undergo clonal evolution and step wise progression to bone marrow failure or transformation to an acute blast phase. Cytogenetic or molecular evidence of clonal evolution usually indicates the onset of an acceleration stage or of transformation to an acute process. An increase in the percentage of blasts in the blood or bone marrow is also an indication of worsening disease. The finding of less than 20 % of blasts in the blood or bone marrow signifies acceleration and more than 20% of blasts is sufficient to diagnose a blast phase.
Leukocytosis , Thrombocytosis, excessive magaryocytic proliferation , myelofibrosis and organomegaly are the features that can overlap among the myeloproliferative diseases and complicate the the diagnosis of specific disease entities. For CML, the BCR/ABL fusion gene, in association with characteristic morphologic and clinical findings permits an unequivocal diagnosis.
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Myeloproliferative disease from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies