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! style="padding: 0 5px; font-size: 80%; background: #A8A8A8;" align=center| {{fontcolor|#2B3B44| | ! style="padding: 0 5px; font-size: 80%; background: #A8A8A8;" align=center| {{fontcolor|#2B3B44|Polycythemia<BR>Resident Survival Guide}} | ||
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! style="font-size: 80%; padding: 0 5px; background: #DCDCDC; border-radius: 5px 5px 5px 5px;" align=left | [[{{PAGENAME}}#Overview|Overview]] | ! style="font-size: 80%; padding: 0 5px; background: #DCDCDC; border-radius: 5px 5px 5px 5px;" align=left | [[{{PAGENAME}}#Overview|Overview]] | ||
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{{SK}} Approach to | {{SK}} Approach to polycythemia, Approach to high hemoglobin level, Approach to high red blood cell count | ||
==Overview== | ==Overview== | ||
Polycythemia is defined as high [[hemoglobin]] (>16.5 g/dl in men and >16 g/dl in women) or [[hematocrit]] level (>49%in men and >48% in women). This elevated level might be due to declining the [[plasma]] volume (relative or spurious polycythemia) or rising the number of [[red blood cells]] (true polycythemia). | |||
==Causes== | ==Causes== | ||
===Life Threatening Causes=== | ===Life Threatening Causes=== | ||
* Critical dehydration owing to fluid loss such as severe [[diarrhea]] or [[vomiting]], which can result in Spurious [[Polycythemia]] | * Critical [[dehydration]] owing to fluid loss such as severe [[diarrhea]] or [[vomiting]], which can result in Spurious [[Polycythemia]] | ||
* Severe [[cyanotic heart diseases]] with [[right-to-left shunt]]s | * Severe [[cyanotic heart diseases]] with [[right-to-left shunt]]s | ||
* End-stage [[cancer]] related to [[EPO]]-secreting tumors such as [[hepatocellular carcinoma]], [[parathyroid carcinoma]], [[pheochromocytoma]], or [[renal cell carcinoma]] | * End-stage [[cancer]] related to [[EPO]]-secreting tumors such as [[hepatocellular carcinoma]], [[parathyroid carcinoma]], [[pheochromocytoma]], or [[renal cell carcinoma]] <ref name="McMullinBareford2005">{{cite journal|last1=McMullin|first1=Mary F.|last2=Bareford|first2=D.|last3=Campbell|first3=P.|last4=Green|first4=A. R.|last5=Harrison|first5=Claire|last6=Hunt|first6=Beverley|last7=Oscier|first7=D.|last8=Polkey|first8=M. I.|last9=Reilly|first9=J. T.|last10=Rosenthal|first10=E.|last11=Ryan|first11=Kate|last12=Pearson|first12=T. C.|last13=Wilkins|first13=Bridget|title=Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis|journal=British Journal of Haematology|volume=130|issue=2|year=2005|pages=174–195|issn=0007-1048|doi=10.1111/j.1365-2141.2005.05535.x}}</ref> | ||
===Common Causes=== | ===Common Causes=== | ||
:Primary polycythemia | :Primary [[polycythemia]] <ref name="pmid30252337">{{cite journal |vauthors=Pillai AA, Fazal S, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30252337 |doi= |url=}}</ref> | ||
:* [[Polycythemia vera]] and its complications | :* [[Polycythemia vera]] and its [[complications]] | ||
:Secondary polycythemia | :Secondary [[polycythemia]] | ||
:*[[Chronic lung disease]] | :*[[Chronic lung disease]] | ||
:* [[High altitude]] | :* [[High altitude]] | ||
:* EPO-producing | :* EPO-producing [[tumor]]s | ||
:* High carboxyhemoglobin: mostly observed in smokers | :* High carboxyhemoglobin: mostly observed in smokers | ||
:*Kidney diseases, such as Renal | :*[[Kidney]] [[diseases]], such as [[Renal cyst]]s and [[renal artery stenosis]] | ||
:* Iatrogenic | :* Iatrogenic Causes: | ||
::* [[Steroid]]s | |||
::* [[Erythropoietin]] treatment | |||
::* Anabolic [[testosterone]] replacement therapy | |||
==Diagnosis== | ==Diagnosis== | ||
Shown below is an algorithm summarizing the diagnosis of [[polycythemia]] according to hematology guidelines. | Shown below is an algorithm summarizing the diagnosis of [[polycythemia]] according to hematology guidelines. <ref name="pmid30252337">{{cite journal |vauthors=Pillai AA, Fazal S, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30252337 |doi= |url=}}</ref> <ref name="McMullinBareford2005">{{cite journal|last1=McMullin|first1=Mary F.|last2=Bareford|first2=D.|last3=Campbell|first3=P.|last4=Green|first4=A. R.|last5=Harrison|first5=Claire|last6=Hunt|first6=Beverley|last7=Oscier|first7=D.|last8=Polkey|first8=M. I.|last9=Reilly|first9=J. T.|last10=Rosenthal|first10=E.|last11=Ryan|first11=Kate|last12=Pearson|first12=T. C.|last13=Wilkins|first13=Bridget|title=Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis|journal=British Journal of Haematology|volume=130|issue=2|year=2005|pages=174–195|issn=0007-1048|doi=10.1111/j.1365-2141.2005.05535.x}}</ref> <ref>{{cite book | last = Jameson | first = J | title = Harrison's principles of internal medicine | publisher = McGraw-Hill Education | location = New York | year = 2018 | isbn = 978-1259643996 }}</ref> | ||
'''Abbreviations:''' '''Hgb:''' [[Hemoglobin]], '''[[Hct]]:''' [[Hematocrit]] '''[[RBC]]:''' [[Red blood cell]], '''EPO:''' [[Erythropoietin]], '''[[COPD]]:''' [[Chronic obstructive pulmonary disease]] | |||
{{Family tree/start}} | {{Family tree/start}} | ||
| Line 79: | Line 82: | ||
==Treatment== | ==Treatment== | ||
Shown below is an algorithm summarizing the treatment of [[polycythemia vera]]. | Curing the underlying [[causes]] of secondary polycythemia is the primary way of treatment in this disease. Shown below is an algorithm summarizing the treatment of [[polycythemia vera]]. <ref name="TefferiVannucchi2018">{{cite journal|last1=Tefferi|first1=Ayalew|last2=Vannucchi|first2=Alessandro M.|last3=Barbui|first3=Tiziano|title=Polycythemia vera treatment algorithm 2018|journal=Blood Cancer Journal|volume=8|issue=1|year=2018|issn=2044-5385|doi=10.1038/s41408-017-0042-7}}</ref> <ref name="MarchioliFinazzi2013">{{cite journal|last1=Marchioli|first1=Roberto|last2=Finazzi|first2=Guido|last3=Specchia|first3=Giorgina|last4=Cacciola|first4=Rossella|last5=Cavazzina|first5=Riccardo|last6=Cilloni|first6=Daniela|last7=De Stefano|first7=Valerio|last8=Elli|first8=Elena|last9=Iurlo|first9=Alessandra|last10=Latagliata|first10=Roberto|last11=Lunghi|first11=Francesca|last12=Lunghi|first12=Monia|last13=Marfisi|first13=Rosa Maria|last14=Musto|first14=Pellegrino|last15=Masciulli|first15=Arianna|last16=Musolino|first16=Caterina|last17=Cascavilla|first17=Nicola|last18=Quarta|first18=Giovanni|last19=Randi|first19=Maria Luigia|last20=Rapezzi|first20=Davide|last21=Ruggeri|first21=Marco|last22=Rumi|first22=Elisa|last23=Scortechini|first23=Anna Rita|last24=Santini|first24=Simone|last25=Scarano|first25=Marco|last26=Siragusa|first26=Sergio|last27=Spadea|first27=Antonio|last28=Tieghi|first28=Alessia|last29=Angelucci|first29=Emanuele|last30=Visani|first30=Giuseppe|last31=Vannucchi|first31=Alessandro Maria|last32=Barbui|first32=Tiziano|title=Cardiovascular Events and Intensity of Treatment in Polycythemia Vera|journal=New England Journal of Medicine|volume=368|issue=1|year=2013|pages=22–33|issn=0028-4793|doi=10.1056/NEJMoa1208500}}</ref> <ref name="Quintás-CardamaAbdel-Wahab2013">{{cite journal|last1=Quintás-Cardama|first1=Alfonso|last2=Abdel-Wahab|first2=Omar|last3=Manshouri|first3=Taghi|last4=Kilpivaara|first4=Outi|last5=Cortes|first5=Jorge|last6=Roupie|first6=Anne-Laure|last7=Zhang|first7=Su-Jiang|last8=Harris|first8=David|last9=Estrov|first9=Zeev|last10=Kantarjian|first10=Hagop|last11=Levine|first11=Ross L.|last12=Verstovsek|first12=Srdan|title=Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a|journal=Blood|volume=122|issue=6|year=2013|pages=893–901|issn=0006-4971|doi=10.1182/blood-2012-07-442012}}</ref> <ref name="BarbuiMasciulli2015">{{cite journal|last1=Barbui|first1=Tiziano|last2=Masciulli|first2=Arianna|last3=Marfisi|first3=Maria Rosa|last4=Tognoni|first4=Giovanni|last5=Finazzi|first5=Guido|last6=Rambaldi|first6=Alessandro|last7=Vannucchi|first7=Alessandro|title=White blood cell counts and thrombosis in polycythemia vera: a subanalysis of the CYTO-PV study|journal=Blood|volume=126|issue=4|year=2015|pages=560–561|issn=0006-4971|doi=10.1182/blood-2015-04-638593}}</ref> <ref name="BarbuiVannucchi2017">{{cite journal|last1=Barbui|first1=Tiziano|last2=Vannucchi|first2=Alessandro M.|last3=Carobbio|first3=Alessandra|last4=Rumi|first4=Elisa|last5=Finazzi|first5=Guido|last6=Gisslinger|first6=Heinz|last7=Ruggeri|first7=Marco|last8=Randi|first8=Maria Luigia|last9=Cazzola|first9=Mario|last10=Rambaldi|first10=Alessandro|last11=Gisslinger|first11=Bettina|last12=Pieri|first12=Lisa|last13=Thiele|first13=Juergen|last14=Pardanani|first14=Animesh|last15=Tefferi|first15=Ayalew|title=The effect of arterial hypertension on thrombosis in low-risk polycythemia vera|journal=American Journal of Hematology|volume=92|issue=1|year=2017|pages=E5–E6|issn=03618609|doi=10.1002/ajh.24583}}</ref> | ||
{{familytree/start}} | {{familytree/start}} | ||
{{familytree | | | | | | | | | A01 | | | | | |A01=The main treatment in Polycythemia Vera }} | {{familytree | | | | | | | | | A01 | | | | | |A01=The main treatment in [[Polycythemia Vera]] }} | ||
{{familytree | | | | | | | | | |!| | | | | | | | }} | {{familytree | | | | | | | | | |!| | | | | | | | }} | ||
{{familytree | | | | | | | | | B01 | | | | | |B01=Phlebotomy in order to keep hematocrit lower than 45% and prescribe Aspirin(40-100 mg) once every day }} | {{familytree | | | | | | | | | B01 | | | | | |B01=[[Phlebotomy]] in order to keep [[hematocrit]] lower than 45% and prescribe [[Aspirin]](40-100 mg) once every day }} | ||
{{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }} | {{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }} | ||
{{familytree | | C01 | | | | | | | | | | | |C02|C01=Low-risk patient (without any history of thrombosis and ≤60 years)|C02= High-risk patient (with a history of thrombosis or older than 60 years )}} | {{familytree | | C01 | | | | | | | | | | | |C02|C01=Low-risk [[patient]] (without any history of [[thrombosis]] and ≤60 years)|C02= High-risk [[patient]] (with a history of [[thrombosis]] or older than 60 years )}} | ||
{{familytree | | |!| | | | | | | | | | | | | |!| }} | {{familytree | | |!| | | | | | | | | | | | | |!| }} | ||
{{familytree | | D01 | | | | | | | | | | | | D02 |-|-|-|-|-|-|-|-| D03 | | | D01=If a patient has microvascular symptoms, which have not been controlled sufficiently or leukocytosis or cardiovascular symptoms specifically hypertension|D02=Add hydroxyurea with the initial dose of 500 mg twice daily|D03=If the patient could not tolerate | {{familytree | | D01 | | | | | | | | | | | | D02 |-|-|-|-|-|-|-|-|-| D03 | | | D01=If a [[patient]] has [[microvascular]] symptoms, which have not been controlled sufficiently or [[leukocytosis]] or [[cardiovascular]] [[symptoms]] specifically [[hypertension]]|D02=Add [[hydroxyurea]] with the initial dose of 500 mg twice daily|D03=If the [[patient]] could not tolerate [[hydroxyurea]] or was resistant to it}} | ||
{{familytree | | |!| | | | | | | | | |,|-|-|-|^|-|-|-|.| | | | | | |!| | | | }} | {{familytree | | |!| | | | | | | | | |,|-|-|-|^|-|-|-|.| | | | | | | |!| | | | }} | ||
{{familytree | | E01 | | | | | | | | E02 | | | | | |E04| | | | | | E05 | | | |E01=Prescribe Aspirin BID|E02=If the patient has a history of arterial thrombosis|E04=If the patient has a history of venous trombosis|E05=prescribe Pegylated IFN-α or Busulfan}} | {{familytree | | E01 | | | | | | | | E02 | | | | | |E04| | | | | |E05| | | |E01=Prescribe [[Aspirin]] BID|E02=If the [[patient]] has a history of [[arterial thrombosis]]|E04=If the [[patient]] has a history of [[venous]] [[trombosis]]|E05=prescribe Pegylated IFN-α or Busulfan}} | ||
{{familytree | | | | | | | | | | | | |!| | | | | | | |!| | }} | {{familytree | | | | | | | | | | | | |!| | | | | | | |!| | }} | ||
{{familytree | | | | | | | | | | | | F01 | | | | | | F03 |F01=Prescribe Aspirin BID | {{familytree | | | | | | | | | | | | F01 | | | | | | F03 |F01=Prescribe [[Aspirin]] BID|F03=Add systemic [[anticoagulant]] therapy}} | ||
{{familytree/end}} | {{familytree/end}} | ||
==Do's== | ==Do's== | ||
* First, you should differentiate between absolute [[polycythemia]] and relative polycythemia | * First, you should differentiate between absolute [[polycythemia]] and relative [[polycythemia]] | ||
* Ask the patient about using | * Ask the patient about using [[diuretic]]s and products athletes use to improve their function | ||
* Low-dose of Aspirin must be prescribed for all cases of polycythemia vera who do not have any contraindication | * Low-dose of [[Aspirin]] must be prescribed for all cases of [[polycythemia vera]] who do not have any contraindication <ref name="pmid9136963">{{cite journal |vauthors=van Genderen PJ, Mulder PG, Waleboer M, van de Moesdijk D, Michiels JJ |title=Prevention and treatment of thrombotic complications in essential thrombocythaemia: efficacy and safety of aspirin |journal=Br. J. Haematol. |volume=97 |issue=1 |pages=179–84 |date=April 1997 |pmid=9136963 |doi=10.1046/j.1365-2141.1997.d01-2127.x |url=}}</ref> <ref name="McMullinBareford2005">{{cite journal|last1=McMullin|first1=Mary F.|last2=Bareford|first2=D.|last3=Campbell|first3=P.|last4=Green|first4=A. R.|last5=Harrison|first5=Claire|last6=Hunt|first6=Beverley|last7=Oscier|first7=D.|last8=Polkey|first8=M. I.|last9=Reilly|first9=J. T.|last10=Rosenthal|first10=E.|last11=Ryan|first11=Kate|last12=Pearson|first12=T. C.|last13=Wilkins|first13=Bridget|title=Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis|journal=British Journal of Haematology|volume=130|issue=2|year=2005|pages=174–195|issn=0007-1048|doi=10.1111/j.1365-2141.2005.05535.x}}</ref> | ||
==Don'ts== | ==Don'ts== | ||
*Do not analyze JAK2 mutation in every case as a first step when there is no clinical finding related to polycythemia vera | *Do not analyze [[JAK2]] mutation in every case as a first step when there is no clinical finding related to [[polycythemia vera]] | ||
*In women with polycythemia vera do not consider pregnancy as a contraindication | *In women with [[polycythemia vera]] do not consider [[pregnancy]] as a contraindication | ||
*Aspirin should not be prescribed in cases of acquired von Willebrand disease | *[[Aspirin]] should not be prescribed in cases of acquired [[von Willebrand disease]] <ref name="MazeKazi2019">{{cite journal|last1=Maze|first1=Dawn|last2=Kazi|first2=Sajida|last3=Gupta|first3=Vikas|last4=Malinowski|first4=Ann Kinga|last5=Fazelzad|first5=Rouhi|last6=Shah|first6=Prakesh S.|last7=Shehata|first7=Nadine|title=Association of Treatments for Myeloproliferative Neoplasms During Pregnancy With Birth Rates and Maternal Outcomes|journal=JAMA Network Open|volume=2|issue=10|year=2019|pages=e1912666|issn=2574-3805|doi=10.1001/jamanetworkopen.2019.12666}}</ref> <ref name="TefferiBarbui2017">{{cite journal|last1=Tefferi|first1=Ayalew|last2=Barbui|first2=Tiziano|title=Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management|journal=American Journal of Hematology|volume=92|issue=1|year=2017|pages=94–108|issn=03618609|doi=10.1002/ajh.24607}}</ref> | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Projects]] | [[Category:Projects]] | ||
[[Category:Resident survival guide]] | [[Category:Resident survival guide]] | ||
[[Category: | [[Category:Up-to-date]] | ||
Latest revision as of 13:53, 30 September 2020
| Polycythemia Resident Survival Guide |
|---|
| Overview |
| Causes |
| Diagnosis |
| Treatment |
| Do's |
| Don'ts |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alieh Behjat, M.D.[2]
Synonyms and keywords: Approach to polycythemia, Approach to high hemoglobin level, Approach to high red blood cell count
Overview
Polycythemia is defined as high hemoglobin (>16.5 g/dl in men and >16 g/dl in women) or hematocrit level (>49%in men and >48% in women). This elevated level might be due to declining the plasma volume (relative or spurious polycythemia) or rising the number of red blood cells (true polycythemia).
Causes
Life Threatening Causes
- Critical dehydration owing to fluid loss such as severe diarrhea or vomiting, which can result in Spurious Polycythemia
- Severe cyanotic heart diseases with right-to-left shunts
- End-stage cancer related to EPO-secreting tumors such as hepatocellular carcinoma, parathyroid carcinoma, pheochromocytoma, or renal cell carcinoma [1]
Common Causes
- Primary polycythemia [2]
- Polycythemia vera and its complications
- Secondary polycythemia
- Chronic lung disease
- High altitude
- EPO-producing tumors
- High carboxyhemoglobin: mostly observed in smokers
- Kidney diseases, such as Renal cysts and renal artery stenosis
- Iatrogenic Causes:
- Steroids
- Erythropoietin treatment
- Anabolic testosterone replacement therapy
Diagnosis
Shown below is an algorithm summarizing the diagnosis of polycythemia according to hematology guidelines. [2] [1] [3]
Abbreviations: Hgb: Hemoglobin, Hct: Hematocrit RBC: Red blood cell, EPO: Erythropoietin, COPD: Chronic obstructive pulmonary disease
| Elevated Hgb or Hct | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Assess RBC mass | if normal | Relative erythrocytosis | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| If high level | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Measure EPO level | If low | Polycythemia vera | Check JAK2 mutation to confirm | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| If high level | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Assess arterial O2 saturation | If low | Assess cardiac or pulmunary diseases, such as right to left shunts, COPD, high altitute | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| If normal | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Is the paitient smoker? | If no | Measure Hgb O2 affinity | If normal | Diagnostic evaluation for finding tumor producing EPO: Kidney sonography, Brain CT, Abdominopelvic Ct scan | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| If incresed | High oxigen affinity hemoglobinopathy | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| If yes | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Evaluate carboxihemoglobin levels | If normal | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| If high | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Smoker's polycythemia | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Treatment
Curing the underlying causes of secondary polycythemia is the primary way of treatment in this disease. Shown below is an algorithm summarizing the treatment of polycythemia vera. [4] [5] [6] [7] [8]
| The main treatment in Polycythemia Vera | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Phlebotomy in order to keep hematocrit lower than 45% and prescribe Aspirin(40-100 mg) once every day | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Low-risk patient (without any history of thrombosis and ≤60 years) | High-risk patient (with a history of thrombosis or older than 60 years ) | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| If a patient has microvascular symptoms, which have not been controlled sufficiently or leukocytosis or cardiovascular symptoms specifically hypertension | Add hydroxyurea with the initial dose of 500 mg twice daily | If the patient could not tolerate hydroxyurea or was resistant to it | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Prescribe Aspirin BID | If the patient has a history of arterial thrombosis | If the patient has a history of venous trombosis | prescribe Pegylated IFN-α or Busulfan | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Prescribe Aspirin BID | Add systemic anticoagulant therapy | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Do's
- First, you should differentiate between absolute polycythemia and relative polycythemia
- Ask the patient about using diuretics and products athletes use to improve their function
- Low-dose of Aspirin must be prescribed for all cases of polycythemia vera who do not have any contraindication [9] [1]
Don'ts
- Do not analyze JAK2 mutation in every case as a first step when there is no clinical finding related to polycythemia vera
- In women with polycythemia vera do not consider pregnancy as a contraindication
- Aspirin should not be prescribed in cases of acquired von Willebrand disease [10] [11]
References
- ↑ 1.0 1.1 1.2 McMullin, Mary F.; Bareford, D.; Campbell, P.; Green, A. R.; Harrison, Claire; Hunt, Beverley; Oscier, D.; Polkey, M. I.; Reilly, J. T.; Rosenthal, E.; Ryan, Kate; Pearson, T. C.; Wilkins, Bridget (2005). "Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis". British Journal of Haematology. 130 (2): 174–195. doi:10.1111/j.1365-2141.2005.05535.x. ISSN 0007-1048.
- ↑ 2.0 2.1 Pillai AA, Fazal S, Babiker HM. PMID 30252337. Missing or empty
|title=(help) - ↑ Jameson, J (2018). Harrison's principles of internal medicine. New York: McGraw-Hill Education. ISBN 978-1259643996.
- ↑ Tefferi, Ayalew; Vannucchi, Alessandro M.; Barbui, Tiziano (2018). "Polycythemia vera treatment algorithm 2018". Blood Cancer Journal. 8 (1). doi:10.1038/s41408-017-0042-7. ISSN 2044-5385.
- ↑ Marchioli, Roberto; Finazzi, Guido; Specchia, Giorgina; Cacciola, Rossella; Cavazzina, Riccardo; Cilloni, Daniela; De Stefano, Valerio; Elli, Elena; Iurlo, Alessandra; Latagliata, Roberto; Lunghi, Francesca; Lunghi, Monia; Marfisi, Rosa Maria; Musto, Pellegrino; Masciulli, Arianna; Musolino, Caterina; Cascavilla, Nicola; Quarta, Giovanni; Randi, Maria Luigia; Rapezzi, Davide; Ruggeri, Marco; Rumi, Elisa; Scortechini, Anna Rita; Santini, Simone; Scarano, Marco; Siragusa, Sergio; Spadea, Antonio; Tieghi, Alessia; Angelucci, Emanuele; Visani, Giuseppe; Vannucchi, Alessandro Maria; Barbui, Tiziano (2013). "Cardiovascular Events and Intensity of Treatment in Polycythemia Vera". New England Journal of Medicine. 368 (1): 22–33. doi:10.1056/NEJMoa1208500. ISSN 0028-4793.
- ↑ Quintás-Cardama, Alfonso; Abdel-Wahab, Omar; Manshouri, Taghi; Kilpivaara, Outi; Cortes, Jorge; Roupie, Anne-Laure; Zhang, Su-Jiang; Harris, David; Estrov, Zeev; Kantarjian, Hagop; Levine, Ross L.; Verstovsek, Srdan (2013). "Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a". Blood. 122 (6): 893–901. doi:10.1182/blood-2012-07-442012. ISSN 0006-4971.
- ↑ Barbui, Tiziano; Masciulli, Arianna; Marfisi, Maria Rosa; Tognoni, Giovanni; Finazzi, Guido; Rambaldi, Alessandro; Vannucchi, Alessandro (2015). "White blood cell counts and thrombosis in polycythemia vera: a subanalysis of the CYTO-PV study". Blood. 126 (4): 560–561. doi:10.1182/blood-2015-04-638593. ISSN 0006-4971.
- ↑ Barbui, Tiziano; Vannucchi, Alessandro M.; Carobbio, Alessandra; Rumi, Elisa; Finazzi, Guido; Gisslinger, Heinz; Ruggeri, Marco; Randi, Maria Luigia; Cazzola, Mario; Rambaldi, Alessandro; Gisslinger, Bettina; Pieri, Lisa; Thiele, Juergen; Pardanani, Animesh; Tefferi, Ayalew (2017). "The effect of arterial hypertension on thrombosis in low-risk polycythemia vera". American Journal of Hematology. 92 (1): E5–E6. doi:10.1002/ajh.24583. ISSN 0361-8609.
- ↑ van Genderen PJ, Mulder PG, Waleboer M, van de Moesdijk D, Michiels JJ (April 1997). "Prevention and treatment of thrombotic complications in essential thrombocythaemia: efficacy and safety of aspirin". Br. J. Haematol. 97 (1): 179–84. doi:10.1046/j.1365-2141.1997.d01-2127.x. PMID 9136963.
- ↑ Maze, Dawn; Kazi, Sajida; Gupta, Vikas; Malinowski, Ann Kinga; Fazelzad, Rouhi; Shah, Prakesh S.; Shehata, Nadine (2019). "Association of Treatments for Myeloproliferative Neoplasms During Pregnancy With Birth Rates and Maternal Outcomes". JAMA Network Open. 2 (10): e1912666. doi:10.1001/jamanetworkopen.2019.12666. ISSN 2574-3805.
- ↑ Tefferi, Ayalew; Barbui, Tiziano (2017). "Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 92 (1): 94–108. doi:10.1002/ajh.24607. ISSN 0361-8609.