Chronic Myeloid Neoplasms
Myeloproliferative neoplasms (MPN)
Polycythemia vera

Author: Erika M. Baardsen, D.O. and Ling Zhang, M.D. (see Authors page)

Revised: 18 October 2017, last major update October 2017

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: Polycythemia vera [title]
Cite this page: Baardsen, E.M. and Zhang, L. Polycythemia vera. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/myeloproliferativePV.html. Accessed October 23rd, 2017.
Definition / general
  • Also referred to as polycythemia rubra vera, primary polycythemia and essential polycythemia
  • BCR-ABL1 negative myeloproliferative neoplasm (MPN) characterized by peripheral erythrocytosis with or without leukocytosis or thrombocytosis, splenomegaly, thrombotic episodes, bleeding, pruritus, plethora, symptoms related to altered microcirculation and potential myelofibrosis or leukemic transformation due to increased red blood cell production independent of mechanisms that typically regulate erythropoiesis
Essential features
  • Relatively common MPN characterized by increased red blood cell production with elevated red blood cell count and hemoglobin level, which is not proportional to erythropoietin (EPO) production (marked decreased EPO level)
  • Diagnosis requires all 3 major criteria or 2 major and minor criterion (see Diagnosis section below, 2016 WHO classification)
  • JAK2V617F (exon 14, valine to phenylalanine) and JAK2 exon 12 mutations are identified in > 95% and approximately 5% of patients, respectively (Blood 2006;108:1865, Hum Pathol 2006;37:1458, N Engl J Med 2007;356:459)
  • Three phases:
    • Prodromal / pre-polycythemic
    • Overt Polycythemic
    • Post Polycythemic Myelofibrosis / Spent (20%)
  • There is a minor subset of PV patients (< 10%) in post polycythemic phase with blastic transformation
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
Diagnosis
Diagnostic Criteria for Post-Polycythemic Myelofibrosis (post-PV MF):
Laboratory
Prognostic factors
Case reports
Clinical Outcome and Treatment:
Microscopic (histologic) description
Microscopic (histologic) images

Images hosted on PathOut server:

Peripheral blood:

Hb of 19 g/dL, WBC of 12.8

Spent phase



Bone marrow biopsy:

Markedly hypercellular marrow with prominent megakaryocytes

Posttreatment with myelofibrosis due to metastatic prostate carcinom


Left two images: predominance of immature cells, some erythroid precursors have dysplastic features; right: marked increase in coarse reticulin fibers (reticulin stain)



Images hosted on other servers:

Bone marrow biopsy:

Various images

Peripheral smear description
Positive stains
Negative stains
Molecular / cytogenetics description
Differential diagnosis
Additional references
Board review question #1
Board review answer #1
Board review question #2
Board review answer #2