Pathogenesis of polycythemia vera—new concepts

Golde, David W.; Cline, Martin J.

doi:10.1002/ajh.2830010311

Cited by 5 publications

(1 citation statement)

References 19 publications

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“…We aimed to investigate the mechanism(s) regulating erythroid cell population size with the aim of throwing light on the pathogenesis of Polycythemia vera (PV), an acquired, chronic, clonal, myeloproliferative/myeloaccumulative disorder [1 -3]. PV is typically characterized by trilineal marrow hyperplasia in which the major clinicopathological emphasis is on the erythroid lineage [4,5], and in which a defect in control of erythroid cell population size is intrinsic to the cells [2]. In this condition, a relentless increase in red cell number occurs in the presence of normal O 2 saturation [6], while levels of circulating erythropoietin (Epo), the key hormone controlling erythropoiesis during normal adult life [7], are often depressed [8,9].…”

Section: Introductionmentioning

confidence: 99%

Overexpression of SOCS-2 and SOCS-3 genes reverses erythroid overgrowth and IGF-I hypersensitivity of primary polycythemia vera (PV) cells

Usenko

Eskinazi

Correa

et al. 2007

Leukemia & Lymphoma

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Polycythemia vera (PV), an acquired, chronic, clonal disorder arising in a multipotential hematopoietic progenitor cell, is characterized by hyperplasia of three major myeloid lineages, with a pronounced increase in cells of the erythroid lineage. Erythroid progenitor cells in PV are strikingly hypersensitive to insulin-like growth factor-I (IGF-I); this effect is specific and is mediated through the IGF-I receptor. To investigate the possibility that in PV the increase in number of erythroid progenitors and their hypersensitivity to IGF-I result from a defect in negative regulation of cytokine activity, we examined the expression of members of the SOCS gene family. Circulating mononuclear cells, grown in serum-free methylcellulose medium in the presence of IGF-I, produced BFU-E-derived colonies whose cells revealed a reduction of SOCS-2 and SOCS-3 expression in PV only. Overexpression of these genes in transfected PV cells reduced their erythroid overgrowth and IGF-I hypersensitivity. We hypothesize that a defect in expression of SOCS-2 and SOCS-3 genes may be crucial for the IGF-I hypersensitivity and progressive increase in erythroid cell population size characteristic of PV.

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Section: Introductionmentioning

confidence: 99%

Overexpression of SOCS-2 and SOCS-3 genes reverses erythroid overgrowth and IGF-I hypersensitivity of primary polycythemia vera (PV) cells

Usenko

Eskinazi

Correa

et al. 2007

Leukemia & Lymphoma

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Zur Frage der Existenz eines myeloproliferativen Faktors bei Patienten mit myeloproliferativem Syndrom

Essers¹,

Nowak²

1979

Blut

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Serum of patients suffering from a chronic myeloproliferative disorder (polycythaemia, era, osteomyelofibrosis, chronic myeloid leukaemia) and serum of lethally irradiated rats injected before application of a single doses of erythropoietin did not enhance the effect of erythropoietin -- measured with the iron incorporation rate of polycythemic mice. The rationale for these experiments is to try to find a "myeloproliferative factor", which augments the number of stem cells as described in sera of patients with polycythaemia vera, osteomyelofibrosis, and lethally irradiated mice.

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Erythropoiesis in Familial Erythrocytosis

Greenberg¹,

Golde²

1977

N Engl J Med

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We found primary erythrocytosis in two male siblings with hematologically normal parents. To clarify the abnormalities in erythropoiesis, we studied erythropoietin production in the older sibling as well as in vivo and in vitro responses of bone marrow to various stimuli. His erythropoietin excretion after a 1000-ml phlebotomy increased by 0 to 11 units per day. In liquid-suspension culture, erythropoiesis was prominently augmented by erythropoietin and unstimulated erythropoiesis was greater and more prolonged than normal. Numbers of erythroid colonies rose in methylcellulose culture without exogenous erythropoietin, and cloning increased with added erythropoietin. Anti-erythropoietin antibody substantially decreased erythropoiesis in vitro. Increased bone-marrow erythropoiesis was also demonstrated in murine diffusion chambers. The principal abnormality in this familial erythrocytosis appears to be a greatly expanded erythropoietic precursor pool that is responsive to erythropoietin in vitro and in vivo. This abnormality is analogous to the functional erythropoietic defect in typical polycythemia vera.

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Pathogenesis of polycythemia vera—new concepts

Cited by 5 publications

References 19 publications

Overexpression of SOCS-2 and SOCS-3 genes reverses erythroid overgrowth and IGF-I hypersensitivity of primary polycythemia vera (PV) cells

Overexpression of SOCS-2 and SOCS-3 genes reverses erythroid overgrowth and IGF-I hypersensitivity of primary polycythemia vera (PV) cells

Zur Frage der Existenz eines myeloproliferativen Faktors bei Patienten mit myeloproliferativem Syndrom

Erythropoiesis in Familial Erythrocytosis

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