Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase

MedGen UID:: 1641083
•Concept ID:: C3805232
•: Neoplastic Process

Synonyms:	Myelofibrosis due to and following polycythemia vera; Polycythemia Vera, "Spent" Phase; Polycythemia Vera, Post-Polycythemic Myelofibrosis and Myeloid Metaplasia Stage; Post-polycythemia Vera Myelofibrosis; Post-polycythemia vera myelofibrosis; Post-PV MF; Spent Phase and Post-Polycythemic Myelofibrosis
SNOMED CT:	Post-polycythemia vera myelofibrosis (871540009); Myelofibrosis due to and following polycythemia vera (871540009)

Definition

Progression of polycythemia vera characterized by decreased erythropoiesis and granulopoiesis, reticulin and collagen fibrosis of the bone marrow, leucoerythroblastic reaction in the peripheral blood, poikilocytosis, tear-drop shaped red blood cells, and splenomegaly. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPolycythemia Vera, Post-Polycythemic Myelofibrosis Phase

Disorder by Site
- Hematopoietic and Lymphoid System Disorder
  - Hematologic disorder
    - Bone marrow disorder
      - Bone marrow neoplasm
        Acquired polycythemia vera
        Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase

Recent clinical studies

Etiology

Characteristics and clinical significance of cytogenetic abnormalities in polycythemia vera.

Tang G, Hidalgo Lopez JE, Wang SA, Hu S, Ma J, Pierce S, Zuo W, Carballo-Zarate AA, Yin CC, Tang Z, Li S, Medeiros LJ, Verstovsek S, Bueso-Ramos CE
Haematologica 2017 Sep;102(9):1511-1518. Epub 2017 May 4 doi: 10.3324/haematol.2017.165795. PMID: 28473622 Free PMC Article

Neutrophilic leukocytosis in advanced stage polycythemia vera: hematopathologic features and prognostic implications.

Boiocchi L, Gianelli U, Iurlo A, Fend F, Bonzheim I, Cattaneo D, Knowles DM, Orazi A
Mod Pathol 2015 Nov;28(11):1448-57. Epub 2015 Sep 4 doi: 10.1038/modpathol.2015.100. PMID: 26336886

Diagnostic impact of bone marrow histopathology in polycythemia vera (PV).

Thiele J, Kvasnicka HM
Histol Histopathol 2005 Jan;20(1):317-28. doi: 10.14670/HH-20.317. PMID: 15578448

The therapy of myelofibrosis: targeting pathogenesis.

Mesa RA
Int J Hematol 2002 Aug;76 Suppl 2:296-304. doi: 10.1007/BF03165138. PMID: 12430941

Clinical and scientific advances in the Philadelphia-chromosome negative chronic myeloproliferative disorders.

Mesa RA
Int J Hematol 2002 Aug;76 Suppl 2:193-203. doi: 10.1007/BF03165117. PMID: 12430925

See all (5)

Diagnosis

Characteristics and clinical significance of cytogenetic abnormalities in polycythemia vera.

Neutrophilic leukocytosis in advanced stage polycythemia vera: hematopathologic features and prognostic implications.

Boiocchi L, Gianelli U, Iurlo A, Fend F, Bonzheim I, Cattaneo D, Knowles DM, Orazi A
Mod Pathol 2015 Nov;28(11):1448-57. Epub 2015 Sep 4 doi: 10.1038/modpathol.2015.100. PMID: 26336886

Diagnostic impact of bone marrow histopathology in polycythemia vera (PV).

Thiele J, Kvasnicka HM
Histol Histopathol 2005 Jan;20(1):317-28. doi: 10.14670/HH-20.317. PMID: 15578448

The therapy of myelofibrosis: targeting pathogenesis.

Mesa RA
Int J Hematol 2002 Aug;76 Suppl 2:296-304. doi: 10.1007/BF03165138. PMID: 12430941

Clinical and scientific advances in the Philadelphia-chromosome negative chronic myeloproliferative disorders.

Mesa RA
Int J Hematol 2002 Aug;76 Suppl 2:193-203. doi: 10.1007/BF03165117. PMID: 12430925

See all (5)

Prognosis

Characteristics and clinical significance of cytogenetic abnormalities in polycythemia vera.

Neutrophilic leukocytosis in advanced stage polycythemia vera: hematopathologic features and prognostic implications.

Boiocchi L, Gianelli U, Iurlo A, Fend F, Bonzheim I, Cattaneo D, Knowles DM, Orazi A
Mod Pathol 2015 Nov;28(11):1448-57. Epub 2015 Sep 4 doi: 10.1038/modpathol.2015.100. PMID: 26336886

Karyotypic abnormalities in myelofibrosis following polycythemia vera.

Andrieux J, Demory JL, Caulier MT, Agape P, Wetterwald M, Bauters F, Laï JL
Cancer Genet Cytogenet 2003 Jan 15;140(2):118-23. doi: 10.1016/s0165-4608(02)00678-7. PMID: 12645649

Clinical and scientific advances in the Philadelphia-chromosome negative chronic myeloproliferative disorders.

Mesa RA
Int J Hematol 2002 Aug;76 Suppl 2:193-203. doi: 10.1007/BF03165117. PMID: 12430925

See all (4)