Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis

McMullin, Mary Frances; Bareford, David; Campbell, Peter J.; Green, Anthony; Harrison, Claire; Hunt, Beverley J.; Oscier, David; Polkey, MI; Reilly, John T.; Rosenthal, Éric; Ryan, Kate; Pearson, Thomas C.; Wilkins, Bridget S.

doi:10.1111/j.1365-2141.2005.05535.x

Cited by 317 publications

(259 citation statements)

References 135 publications

(157 reference statements)

Supporting

Mentioning

234

Contrasting

Unclassified

Order By: Relevance

“…22 To date, indications of IFN-a for the treatment of PV and ET have remained generally limited to high-risk patients younger than 40 years as well as to pregnancy in published guidelines. 81,82 Indeed, when cytoreduction is necessary during pregnancy, many publications have documented the safety of IFN-a in MPN mothers as well as fetuses. 98,99 However, there are very limited data available on the long-term safety of IFN-a in children exposed to this drug during pregnancy.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Interferon-α therapy in bcr-abl-negative myeloproliferative neoplasms

2008

View full text Add to dashboard Cite

Interferon (IFN) was the first cytokine discovered 50 years ago, with a wide range of biological properties, including immunomodulatory, proapoptotic and antiangiogenic activities, that rapidly raised interest in its therapeutic use in malignancies. IFN-receptor characterization was also pivotal in the discovery of the JAK/STAT signaling pathway. Among the large IFN family, mainly one of the type I IFN, IFN-a2, is used in therapy. Many clinical trials have shown remarkable efficacy of IFN-a in bcr-abl-negative myeloproliferative neoplasms (MPNs), especially polycythemia vera (PV), and essential thrombocythemia (ET). IFN-a induces about 80% of hematological responses in those diseases and is able to reduce splenomegaly, as well as relieve pruritus and other constitutional symptoms. Yet its use was limited by toxicity, leading to early treatment discontinuation in about 20% of the patients. However, its lack of leukemogenic potential and its possible use during pregnancy have already made IFN-a the drug of choice for younger MPN patients. In addition, several studies have shown a probably selective effect of IFN-a on PV and ET clones, as shown by cytogenetic remissions, reversions to polyclonal hematopoiesis, and more recently by induction of JAK2V617F complete molecular remissions in PV which may widen the indications of IFN-a in JAK2-mutated MPN.

show abstract

Section: Discussionmentioning

confidence: 99%

“…Those findings have however led published guidelines in ET and PV to recommend the use of clearly non-leukemogenic drugs such as IFN-a in younger patients requiring cytoreductive therapy. 81,82 Has IFN-a a selective effect on myeloproliferative clones?…”

Section: Ifn-a and Leukemic Evolutionmentioning

confidence: 99%

Interferon-α therapy in bcr-abl-negative myeloproliferative neoplasms

2008

View full text Add to dashboard Cite

show abstract

“…In severe COPD, polycythemia should be recognized and managed with institution or adjustment of LTOT, aspirin and appropriate venesection when the hematocrit exceeds 0.56 [70], in order to prevent thrombotic events due to increased blood viscosity.…”

Section: Hematological Coagulopathy Polycythemia and Anemiamentioning

confidence: 99%

Extrapulmonary comorbidities in chronic obstructive pulmonary disease: state of the art

Patel

Hurst

2011

Expert Review of Respiratory Medicine

138

108

View full text Add to dashboard Cite

“…A total of 181 patients, who did not fulfil the polycythemia vera (PV) diagnostic criteria proposed by the British Committee for Standards in Hematology, 18 and had a raised red cell mass, have been investigated. All patients provided informed written consent on entering the study, which had been approved by the Queen's University Research Ethics Committee, in accordance with the Declaration of Helsinki.…”

Section: Patientsmentioning

confidence: 99%

A novel erythrocytosis-associated PHD2 mutation suggests the location of a HIF binding groove

Percy

Furlow

Beer

et al. 2007

Blood

139

View full text Add to dashboard Cite

The molecular basis of the erythrocytosis group of red cell disorders is incompletely defined. Some cases are due to dysregulation of erythropoietin (Epo) synthesis. The hypoxia inducible transcription factor (HIF) tightly regulates Epo synthesis. HIF in turn is regulated through its ␣ subunit, which under normoxic conditions is hydroxylated on specific prolines and targeted for degradation by the von Hippel Lindau (VHL) protein. Several mutations in VHL have been reported in erythrocytosis, but only 1 mutation in the HIF prolyl hydroxylase PHD2 (prolyl hydroxylase domain protein 2) has been described. Here, we report a novel PHD2 mutation, Arg371His, which causes decreased HIF binding, HIF hydroxylase, and HIF inhibitory activities. In the tertiary structure of PHD2, Arg371 lies close to the previously described Pro317Arg mutation site. These findings substantiate PHD2 as a critical enzyme controlling HIF and therefore Epo in humans, and furthermore suggest the location of an active site groove in PHD2 that binds HIF. (Blood.

show abstract

Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis

Cited by 317 publications

References 135 publications

Interferon-α therapy in bcr-abl-negative myeloproliferative neoplasms

Interferon-α therapy in bcr-abl-negative myeloproliferative neoplasms

Extrapulmonary comorbidities in chronic obstructive pulmonary disease: state of the art

A novel erythrocytosis-associated PHD2 mutation suggests the location of a HIF binding groove

Contact Info

Product

Resources

About