Polycythemia resident survival guide: Difference between revisions

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! 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]]
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! style="font-size: 80%; padding: 0 5px; background: #DCDCDC; border-radius: 5px 5px 5px 5px;" align=left | [[{{PAGENAME}}#Causes|Causes]]
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! style="font-size: 80%; padding: 0 5px; background: #DCDCDC; border-radius: 5px 5px 5px 5px;" align=left | [[{{PAGENAME}}#Diagnosis|Diagnosis]]
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! style="font-size: 80%; padding: 0 5px; background: #DCDCDC; border-radius: 5px 5px 5px 5px;" align=left | [[{{PAGENAME}}#Treatment|Treatment]]
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! style="font-size: 80%; padding: 0 5px; background: #DCDCDC; border-radius: 5px 5px 5px 5px;" align=left | [[{{PAGENAME}}#Do's|Do's]]
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! style="font-size: 80%; padding: 0 5px; background: #DCDCDC; border-radius: 5px 5px 5px 5px;" align=left | [[{{PAGENAME}}#Don'ts|Don'ts]]
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__NOTOC__
__NOTOC__
{{Resident survival guide project}}
{{CMG}}; {{AE}} {{ABehjat}}


{{WikiDoc CMG}}; {{AE}} {{ABehjat}}
 
{{SK}} Approach to polycythemia, Approach to high hemoglobin level, Approach to high red blood cell count
==Overview==
==Overview==
[[Polycythemia]] is defined as increasing the [[hemoglobin]] (>16.5 g/dl in men or >16 g/dl in women) or [[hematocrit]] level (>49%in men or >48% in women).<ref name="pmid29426921">{{cite journal| author=Barbui T, Thiele J, Gisslinger H, Kvasnicka HM, Vannucchi AM, Guglielmelli P | display-authors=etal| title=The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion. | journal=Blood Cancer J | year= 2018 | volume= 8 | issue= 2 | pages= 15 | pmid=29426921 | doi=10.1038/s41408-018-0054-y | pmc=5807384 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29426921  }}</ref> This elevated level might be due to declining the plasma volume (relative or spurious [[polycythemia]]) or rising the number of [[red blood cell]]s (true [[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 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 shunts
* 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<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>
* 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 tumors
:* EPO-producing [[tumor]]s
:* High carboxyhemoglobin: mostly observed in smokers
:* High carboxyhemoglobin: mostly observed in smokers
:*Kidney diseases, such as Renal cysts and renal artery stenosis,
:*[[Kidney]] [[diseases]], such as [[Renal cyst]]s and [[renal artery stenosis]]
:* Iatrogenic reasons: steroids, erythropoietin treatment, anabolic testosterone replacement therapy. 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). <ref name="pmid30252337">{{cite journal |vauthors=Pillai AA, Fazal S, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30252337 |doi= |url=}}</ref>
:* Iatrogenic Causes:
::* [[Steroid]]s
::* [[Erythropoietin]] treatment
::* Anabolic [[testosterone]] replacement therapy


==Diagnosis==
==Diagnosis==
Shown below is an algorithm summarizing the diagnosis of [[polycythemia]] according the 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>
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}}
{{Family tree | | | | A01 | | | |A01= Elevated Hgb or Hct}}
{{Family tree | | | | A01 | | | |A01= Elevated Hgb or Hct}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | A01 |-|-|-| A02 |-|-|-| A03 | |A01= Assess RBC mass| A02= if normal| |A03= Relative erythrocytosis}}
{{Family tree | | | | A01 |-|-|-| A02 |-|-|-| A03 | |A01= Assess [[RBC]] mass| A02= if normal| |A03= Relative [[erythrocytosis]]}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | A01 | | | |A01= If High level}}
{{Family tree | | | | A01 | | | |A01= If high level}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | B01 |-|-|-| B02 |-|-|-| B03 |-|-|-| B04 | |B01=  Measure EPO level| B02= If Low| |B03= Polycythemia vera| |B04= Check JAK2 mutation to confirm}}
{{Family tree | | | | B01 |-|-|-| B02 |-|-|-| B03 |-|-|-| B04 | |B01=  Measure EPO level| B02= If low| |B03= [[Polycythemia vera]]| |B04= Check [[JAK2]] mutation to confirm}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | B01 | | | |B01= If High level}}
{{Family tree | | | | B01 | | | |B01= If high level}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | B01|-|-|-| A02 |-|-|-| A03 | |B01= Assess arterial O2 saturation| A02= If low| |A03= Assess cardiac or pulmunary diseases, such as right to left shunts, COPD, high altitute}} | | | |}}
{{Family tree | | | | B01|-|-|-| A02 |-|-|-| A03 | |B01= Assess arterial [[O2 saturation]]| A02= If low| |A03= Assess cardiac or [[pulmunary diseases]], such as [[right to left shunt]]s, [[COPD]], high altitute}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | A01 | | | |A01= If normal}}
{{Family tree | | | | A01 | | | |A01= If normal}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | B01|-|-|-| A02 |-|-|-| A03|-|-|-| A04|-|-|-| A05 | |B01= Is the paitient smoker?| A02= If no| |A03=  Measure Hgb O2 affinity| A04=If normal| A05=Diagnostic evaluation for finding tumor producing EPO: Kidney sonography, Brain CT, Abdominopelvic Ct scan}} | | | |}}
{{Family tree | | | | B01|-|-|-| A02 |-|-|-| A03|-|-|-| A04|-|-|-| A05 | |B01= Is the paitient smoker?| A02= If no| |A03=  Measure Hgb O2 affinity| A04=If normal| A05=Diagnostic evaluation for finding tumor producing EPO: [[Kidney]] [[sonography]], [[Brain CT]], Abdominopelvic [[Ct scan]]}}  
{{Family tree | | | | |!| | | | | | | | | | |!|!|}}
{{Family tree | | | | |!| | | | | | | | | | |!|!|}}
{{Family tree | | | | |!| | | | | | | | | | |!|C01 |-|-| C02 | |C01=If incresed|C02=High oxigen affinity hemoglobinopathy| | | |}}
{{Family tree | | | | |!| | | | | | | | | | |!|C01 |-|-| C02 | |C01=If incresed|C02=High oxigen affinity [[hemoglobinopathy]]}}
{{Family tree | | | | A01 | | | | | | | | | |!| |A01= If yes}}
{{Family tree | | | | A01 | | | | | | | | | |!| |A01= If yes}}
{{Family tree | | | | |!| | | | | | | | | | |!| |}}
{{Family tree | | | | |!| | | | | | | | | | |!| |}}
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{{Family tree | | | | A01 |-|-|-|-|-|-|-|A02| |A01= Evaluate carboxihemoglobin levels|A02= If normal}}
{{Family tree | | | | A01 |-|-|-|-|-|-|-|A02| |A01= Evaluate carboxihemoglobin levels|A02= If normal}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | A01 | | | |A01= If High}}
{{Family tree | | | | A01 | | | |A01= If high}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | | | A01 | | | |A01= Smoker's polycythemia}}
{{Family tree | | | | A01 | | | |A01= Smoker's [[polycythemia]]}}
{{Family tree/end}}
{{Family tree/end}}


==Treatment==
==Treatment==
Shown below is an algorithm summarizing the treatment of polycythemia according to new guidelines.
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 |summary=PE diagnosis Algorithm.}}
 
{{familytree | | | | | | | | A01 |A01= }}  
 
{{familytree | | | | |,|-|-|-|^|-|-|-|-|.| | | }}
 
{{familytree | | | B01 | | | | | | | | B02 | | |B01= |B02= }}
 
{{familytree | | | |!| | | | | | | | | |!| }}
 
{{familytree | | | C01 | | | | | | | | |!| |C01= }}
 
{{familytree | |,|-|^|.| | | | | | | | |!| }}
{{familytree/start}}
{{familytree | D01 | | D02 | | | | | | D03 |D01= |D02= |D03= }}
{{familytree | | | | | | | | | A01 | | | | | |A01=The main treatment in [[Polycythemia Vera]] }}
{{familytree | |!| | | | | | | | | |,|-|^|.| }}
{{familytree | | | | | | | | | |!| | | | | | | | }}
{{familytree | E01 | | | | | | | E02 | | | E03 |E01= |E02= |E03= }}
{{familytree | | | | | | | | | B01 | | | | | |B01=[[Phlebotomy]] in order to keep [[hematocrit]] lower than 45% and prescribe [[Aspirin]](40-100 mg) once every day }}
{{familytree | | | | | | | | | | |!| | | | |!| }}
{{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }}
{{familytree | | | | | | | | | | F01 | | | F02 |F01= |F02= }}
{{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 | | 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 | | 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 | | | | | | | | | | | | 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]]
* 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 <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]]
*In women with [[polycythemia vera]] do not consider [[pregnancy]] as a contraindication
*[[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}}


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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

Common Causes

Primary polycythemia [2]
Secondary polycythemia

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

Don'ts

References

  1. ↑ 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. ↑ 2.0 2.1 Pillai AA, Fazal S, Babiker HM. PMID 30252337. Missing or empty |title= (help)
  3. ↑ Jameson, J (2018). Harrison's principles of internal medicine. New York: McGraw-Hill Education. ISBN 978-1259643996.
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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.
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