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| __NOTOC__ | | __NOTOC__ |
| {{Resident survival guide project}} | | {{Hypokalemia}} |
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| {{WikiDoc CMG}}; {{AE}} {{ABehjat}}
| | Taking potassium contained maedication and treating the underlying disease. |
| ==Overview==
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| 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 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>
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| ==Causes==
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| ===Life Threatening Causes===
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| * Critical dehydration owing to fluid loss such as severe diarrhea or vomiting, which can result in Spurious Polycythemia
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| * Severe cyanotic heart diseases with right-to-left shunts
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| * 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>
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| ===Common Causes===
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| :Primary polycythemia
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| :* [[Polycythemia vera]] and its complications
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| :Secondary polycythemia
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| :* [[Chronic lung disease]]
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| :* [[High altitude]]
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| :* EPO-producing tumors
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| :* High carboxyhemoglobin: mostly observed in smokers
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| :*Kidney diseases, such as Renal cysts and renal artery stenosis,
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| :* 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>
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| ==Diagnosis==
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| 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>
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| {{Family tree/start}}
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| {{Family tree | | | | A01 | | | |A01= Elevated Hgb or Hct}}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | A01 |-|-|-| A02 |-|-|-| A03 | |A01= Assess RBC mass| A02= if normal| |A03= Relative erythrocytosis}}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | A01 | | | |A01= If High level}}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | B01 |-|-|-| B02 |-|-|-| B03 |-|-|-| B04 | |B01= Measure EPO level| B02= If Low| |B03= Polycythemia vera| |B04= Check JAK2 mutation to confirm}}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | B01 | | | |B01= If High level}}
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| {{Family tree | | | | |!| | | | | }}
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| {{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}} | | | |}}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | A01 | | | |A01= If normal}}
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| {{Family tree | | | | |!| | | | | }}
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| {{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}} | | | |}}
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| {{Family tree | | | | |!| | | | | | | | | | |!|!|}}
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| {{Family tree | | | | |!| | | | | | | | | | |!|C01 |-|-| C02 | |C01=If incresed|C02=High oxigen affinity hemoglobinopathy| | | |}}
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| {{Family tree | | | | A01 | | | | | | | | | |!| |A01= If yes}}
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| {{Family tree | | | | |!| | | | | | | | | | |!| |}}
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| {{Family tree | | | | |!| | | | | | | | | | |!| |}}
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| {{Family tree | | | | |!| | | | | | | | | | |!| |}}
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| {{Family tree | | | | A01 |-|-|-|-|-|-|-|A02| |A01= Evaluate carboxihemoglobin levels|A02= If normal}}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | A01 | | | |A01= If High}}
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| {{Family tree | | | | |!| | | | | }}
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| {{Family tree | | | | A01 | | | |A01= Smoker's polycythemia}}
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| {{Family tree/end}}
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| ==Treatment==
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| 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>
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| {{familytree/start}}
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| {{familytree | | | | | | | | | A01 | | | | | |A01=The main treatment in Polycythemia Vera }}
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| {{familytree | | | | | | | | | |!| | | | | | | | }}
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| {{familytree | | | | | | | | | B01 | | | | | |B01=Phlebotomy in order to keep hematocrit lower than 45% and prescribe Aspirin(40-100 mg) once every day }}
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| {{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }}
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| {{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 )}}
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| {{familytree | | |!| | | | | | | | | | | | | |!| }}
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| {{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}}
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| {{familytree | | |!| | | | | | | | | |,|-|-|-|^|-|-|-|.| | | | | | |!| | | | }}
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| {{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}}
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| {{familytree | | | | | | | | | | | | |!| | | | | | | |!| | }}
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| {{familytree | | | | | | | | | | | | F01 | | | | | | F03 |F01=Prescribe Aspirin BID|F02=F02|F03=Add systemic anticoagulant therapy}}
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| {{familytree/end}}
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| ==Do's==
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| * Firstly, you should differentiate between absolute polycythemia and relative polycythemia
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| * Ask the patient about using diuretics and products athletes use to improve their function
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| * 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>
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| ==Don'ts==
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| *Do not analyze JAK2 mutation in every case as a first step when there is no clinical finding related to polycythemia vera
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| *In women with polycythemia vera do not consider pregnancy as a contraindication
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| *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>
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| ==References== | | ==References== |
| {{Reflist|2}} | | {{Reflist|2}} |
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