Polycythemia vera differential diagnosis: Difference between revisions

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Revision as of 13:56, 24 August 2018

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]; Shyam Patel [3]

Overview

Polycythemia vera must be differentiated from a variety of other conditions that might cause polycythemia. Polycythemia vera must also be differentiated from secondary polycythemia, which is usually due to chronic hypoxia. Each of these conditions has different etiologies, symptoms, laboratory abnormalities, physical exam findings, and treatments.

Differential Diagnosis of Polycythemia Vera

Polycythemia vera must be differentiated from a variety of other conditions that might cause polycythemia.^[1]^[2]^[3]

Category	Disease	Etiology	Mutation	Clinical manifestations														Laboratory findings						Gold standard disgnosis	Associated findings
				Demography	Symptoms						Signs							CBC				EPO level	PBS
				Demography	Fatigue	Headache	Shortness of breath	Bleeding	Pain	Other symptoms	Appearance	SaO2	Fever	BP	Tenderness	Splenomegaly	Other signs	Hb	RBC	WBC	Plt	EPO level	PBS
Mutational causes	Polycythemia vera (PV)^[4]	Autonomous erythrocyte production	JAK2 mutation > 95%	Mean age >60 years old	+	+	+	±	Erythromelagia Chest pain	Stroke-like symptoms Pruritis Dizziness Visual disturbance	Facial plethora	Nl	−	↑	Painful erythema	+	Lymphadenopathy	↑	↑ RBC mass	Nl to ↑	Nl to ↑	↓	Elevated normochromic, normocytic RBCs Thrombocytosis Rarely immature cells Leukoerythroblastic picture	WHO criteria for PV	Stroke Venous thrombosis Myelofibrosis Acute leukemia
	Chuvash polycythemia^[5]	Hypoxia-sensing disorder	VHL mutation	Russia, Italy <40 years old	+	+	+	±	Arthralgia	Pruritis Dizziness	Facial plethora	↓	−	↑	Painful erythema	+	Lymphadenopathy	↑	↑	Nl	Nl	↑	Elevated normochromic, normocytic RBCs	Molecular genetic testing	Multiple thrombotic events
	Hereditary methemoglobinemia^[6]	Cytochrome b5 reductase deficiency Hemoglobin M disease	Mutations in globin gene	Infants	±	±	±	−	−	−	Cyanosis	Inaccurately Nl	-	Nl	−	−	Altered mental status	↑	↑	Nl	Nl	Nl	NA	RBC enzyme activity + DNA analysis	Usually asymptomatic
	Primary familial and congenital polycythemia^[7]	Autosomal dominant inheritance	EPOR mutation	Very rare	+	+	±	−	−	−	Facial plethora	Nl	-	Nl to ↑	−	−	Altered mental status	↑	↑	Nl	Nl	Nl to ↓	NA	Isolated erythrocytosis + genetic testing	Mild manifestations of hyperviscosity
Category	Disease	Etiology	Mutation	Demography	Fatigue	Headache	Shortness of breath	Bleeding	Pain	Other symptoms	Appearance	SaO2	Fever	BP	Tenderness	Splenomegaly	Other signs	Hb	RBC	WBC	Plt	EPO level	PBS	Gold standard diagnosis	Associated findings
Iatrogenic causes	Smoking^[8]^[9]	Reduced plasma volume Accelerated erythropoiesis	−	Any	−	−	+	−	−	−	Cyanosis	↓	−	Nl	−	−	−	↑	Nl	Nl to ↑	Nl to ↑	Nl to ↓	NA	Clinical manifestation	NA
	Chronic exposure to carbon monoxide^[10]	Occupational exposure	−	Miners, fire fighters	+	+	+	−	−	Dizziness Altered cognition	Cyanosis, flushed cheeks	Nl	−	Nl	−	−	−	↑	↑	Nl	Nl	Nl to ↓	NA	Blood level of carboxyhemoglobin	Concomitant cyanide exposure
	Diuretics^[11]	Reduced plasma volume	-	Any	-	-	-	-	-	Dizziness	Nl	Nl	-	Nl to ↓	-	-	-	↑	Nl	Nl	Nl	Nl to ↓	NA	Clinical manifestation	NA
	Use of androgens or anabolic steroids^[12]^[13]	Reduced plasma volume Accelerated erythropoiesis	-	Athletes	-	+	+	-	-	-	Acne and hirsutism	Nl	-	Nl to ↑	-	-	Gynecomastia	↑	↑	Nl	Nl	↓	NA	Clinical manifestation	Hypogonadism Cardiac hypertrophy
	Self-injection of erythropoietin^[14]	Reduced plasma volume Accelerated erythropoiesis	-	Athletes	-	+	+	-	-	-	Muscular	↓	-	↑	-	-	-	↑	↑	Nl	Nl	↑	NA	Erythropoietin level	MI Stroke Thromboembolic disease antibody-mediated anemia
Secondary causes	Secondary polycythemia due to hypoxemia^[15]	Chronic lung disease Right-to-left cardiac shunts Sleep apnea High altitude	−	Depends on etiology	+	+	+	−	Depends on etiology	Shortness of breath	Cyanosis	↓	−	Nl to ↑	−	−	Crackles Tachypnea Tachycardia	↑	↑	Nl	Nl	↑	NA	Clinical manifestation + imaging	Variable manifestations given the diverse etiologies
	Secondary polycythemia due to erythropoietin producing tumor^[16]	Renal cell carcinoma Hepatocellular carcinoma Cerebellar hemangioblastoma Pheochromocytoma Uterine fibroids	−	Depends on etiology	+	−	−	−	Depends on etiology	Depends on etiology	Chronically ill	Nl	±	Nl to ↑	±	±	Hepatomegaly Bruising	↑/↓	↑/↓	Nl	↑	↑	NA	Clinical manifestation + biopsy	Variable manifestations given the diverse etiologies
	Hereditary hemorrhagic telangiectasia^[17]	Pulmonary arteriovenous malformations	ACVRL1, ENG, GDF2, SMAD4 mutation	Children	-	+	+	GI bleeding	-	Epistaxis Hemoptysis	Multiple telangiectasia on face, extremities, and body	Nl to ↓	-	Nl	-	-	-	↑/↓	↑/↓	Nl	Nl	↑/↓	NA	Clinical criteria + genomic testing	Autosomal dominant Pulmonary hypertension Cerebral AVMs
Category	Disease	Etiology	Mutation	Demography	Fatigue	Headache	Shortness of breath	Bleeding	Pain	Other symptoms	Appearance	SaO2	Fever	BP	Tenderness	Splenomegaly	Other signs	Hb	RBC	WBC	Plt	EPO level	PBS	Gold standard diagnosis	Associated findings

References

↑ Tefferi A, Barbui T (2015). "Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management". Am J Hematol. 90 (2): 162–73. doi:10.1002/ajh.23895. PMID 25611051.
↑ Sanchez S, Ewton A (2006). "Essential thrombocythemia: a review of diagnostic and pathologic features". Arch Pathol Lab Med. 130 (8): 1144–50. doi:10.1043/1543-2165(2006)130[1144:ET]2.0.CO;2. PMID 16879015.
↑ Jabbour E, Kantarjian H (2014). "Chronic myeloid leukemia: 2014 update on diagnosis, monitoring, and management". Am J Hematol. 89 (5): 547–56. doi:10.1002/ajh.23691. PMID 24729196.
↑ Stein, Brady L.; Oh, Stephen T.; Berenzon, Dmitriy; Hobbs, Gabriela S.; Kremyanskaya, Marina; Rampal, Raajit K.; Abboud, Camille N.; Adler, Kenneth; Heaney, Mark L.; Jabbour, Elias J.; Komrokji, Rami S.; Moliterno, Alison R.; Ritchie, Ellen K.; Rice, Lawrence; Mascarenhas, John; Hoffman, Ronald (2015). "Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery ofJAK2 V617F". Journal of Clinical Oncology. 33 (33): 3953–3960. doi:10.1200/JCO.2015.61.6474. ISSN 0732-183X.
↑ Zhou, Amy W.; Knoche, Eric M.; Engle, Elizabeth K.; Ban-Hoefen, Makiko; Kaiwar, Charu; Oh, Stephen T. (2016). "Clinical Improvement with JAK2 Inhibition in Chuvash Polycythemia". New England Journal of Medicine. 375 (5): 494–496. doi:10.1056/NEJMc1600337. ISSN 0028-4793.
↑ Da-Silva SS, Sajan IS, Underwood JP (August 2003). "Congenital methemoglobinemia: a rare cause of cyanosis in the newborn--a case report". Pediatrics. 112 (2): e158–61. PMID 12897322.
↑ Bento C, McMullin MF, Percy M, et al. Primary Familial and Congenital Polycythemia. 2016 Nov 10. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK395975/
↑ Hasselbalch, Hans Carl (2015). "Smoking as a contributing factor for development of polycythemia vera and related neoplasms". Leukemia Research. 39 (11): 1137–1145. doi:10.1016/j.leukres.2015.09.002. ISSN 0145-2126.
↑ Malenica, Maja; Prnjavorac, Besim; Bego, Tamer; Dujic, Tanja; Semiz, Sabina; Skrbo, Selma; Gusic, Amar; Hadzic, Ajla; Causevic, Adlija (2017). "Effect of Cigarette Smoking on Haematological Parameters in Healthy Population". Medical Archives. 71 (2): 132. doi:10.5455/medarh.2017.71.132-136. ISSN 0350-199X.
↑ Wu, P. E.; Juurlink, D. N. (2014). "Carbon monoxide poisoning". Canadian Medical Association Journal. 186 (8): 611–611. doi:10.1503/cmaj.130972. ISSN 0820-3946.
↑ Pollak R, Maddux MS, Cohan J, Jacobsson PK, Mozes MF (March 1988). "Erythrocythemia following renal transplantation: influence of diuretic therapy". Clin. Nephrol. 29 (3): 119–23. PMID 3282731.
↑ Krauss DJ, Taub HA, Lantinga LJ, Dunsky MH, Kelly CM (December 1991). "Risks of blood volume changes in hypogonadal men treated with testosterone enanthate for erectile impotence". J. Urol. 146 (6): 1566–70. PMID 1942342.
↑ Morales A, Johnston B, Heaton JP, Lundie M (March 1997). "Testosterone supplementation for hypogonadal impotence: assessment of biochemical measures and therapeutic outcomes". J. Urol. 157 (3): 849–54. PMID 9072584.
↑ Elliott, S (2008). "Erythropoiesis-stimulating agents and other methods to enhance oxygen transport". British Journal of Pharmacology. 154 (3): 529–541. doi:10.1038/bjp.2008.89. ISSN 0007-1188.
↑ Nadeem, Omar; Gui, Jiang; Ornstein, Deborah L. (2012). "Prevalence of Venous Thromboembolism in Patients With Secondary Polycythemia". Clinical and Applied Thrombosis/Hemostasis. 19 (4): 363–366. doi:10.1177/1076029612460425. ISSN 1076-0296.
↑ Da Silva JL, Lacombe C, Bruneval P, Casadevall N, Leporrier M, Camilleri JP, Bariety J, Tambourin P, Varet B (February 1990). "Tumor cells are the site of erythropoietin synthesis in human renal cancers associated with polycythemia". Blood. 75 (3): 577–82. PMID 2297568.
↑ McDonald J, Pyeritz RE. Hereditary Hemorrhagic Telangiectasia. 2000 Jun 26 [Updated 2017 Feb 2]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1351/

[pmid25611051-1] Tefferi A, Barbui T (2015). "Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management". Am J Hematol. 90 (2): 162–73. doi:10.1002/ajh.23895. PMID 25611051.

[pmid16879015-2] Sanchez S, Ewton A (2006). "Essential thrombocythemia: a review of diagnostic and pathologic features". Arch Pathol Lab Med. 130 (8): 1144–50. doi:10.1043/1543-2165(2006)130[1144:ET]2.0.CO;2. PMID 16879015.

[pmid24729196-3] Jabbour E, Kantarjian H (2014). "Chronic myeloid leukemia: 2014 update on diagnosis, monitoring, and management". Am J Hematol. 89 (5): 547–56. doi:10.1002/ajh.23691. PMID 24729196.

[SteinOh2015-4] Stein, Brady L.; Oh, Stephen T.; Berenzon, Dmitriy; Hobbs, Gabriela S.; Kremyanskaya, Marina; Rampal, Raajit K.; Abboud, Camille N.; Adler, Kenneth; Heaney, Mark L.; Jabbour, Elias J.; Komrokji, Rami S.; Moliterno, Alison R.; Ritchie, Ellen K.; Rice, Lawrence; Mascarenhas, John; Hoffman, Ronald (2015). "Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery ofJAK2 V617F". Journal of Clinical Oncology. 33 (33): 3953–3960. doi:10.1200/JCO.2015.61.6474. ISSN 0732-183X.

[ZhouKnoche2016-5] Zhou, Amy W.; Knoche, Eric M.; Engle, Elizabeth K.; Ban-Hoefen, Makiko; Kaiwar, Charu; Oh, Stephen T. (2016). "Clinical Improvement with JAK2 Inhibition in Chuvash Polycythemia". New England Journal of Medicine. 375 (5): 494–496. doi:10.1056/NEJMc1600337. ISSN 0028-4793.

[pmid12897322-6] Da-Silva SS, Sajan IS, Underwood JP (August 2003). "Congenital methemoglobinemia: a rare cause of cyanosis in the newborn--a case report". Pediatrics. 112 (2): e158–61. PMID 12897322.

[7] Bento C, McMullin MF, Percy M, et al. Primary Familial and Congenital Polycythemia. 2016 Nov 10. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK395975/

[Hasselbalch2015-8] Hasselbalch, Hans Carl (2015). "Smoking as a contributing factor for development of polycythemia vera and related neoplasms". Leukemia Research. 39 (11): 1137–1145. doi:10.1016/j.leukres.2015.09.002. ISSN 0145-2126.

[MalenicaPrnjavorac2017-9] Malenica, Maja; Prnjavorac, Besim; Bego, Tamer; Dujic, Tanja; Semiz, Sabina; Skrbo, Selma; Gusic, Amar; Hadzic, Ajla; Causevic, Adlija (2017). "Effect of Cigarette Smoking on Haematological Parameters in Healthy Population". Medical Archives. 71 (2): 132. doi:10.5455/medarh.2017.71.132-136. ISSN 0350-199X.

[WuJuurlink2014-10] Wu, P. E.; Juurlink, D. N. (2014). "Carbon monoxide poisoning". Canadian Medical Association Journal. 186 (8): 611–611. doi:10.1503/cmaj.130972. ISSN 0820-3946.

[pmid3282731-11] Pollak R, Maddux MS, Cohan J, Jacobsson PK, Mozes MF (March 1988). "Erythrocythemia following renal transplantation: influence of diuretic therapy". Clin. Nephrol. 29 (3): 119–23. PMID 3282731.

[pmid1942342-12] Krauss DJ, Taub HA, Lantinga LJ, Dunsky MH, Kelly CM (December 1991). "Risks of blood volume changes in hypogonadal men treated with testosterone enanthate for erectile impotence". J. Urol. 146 (6): 1566–70. PMID 1942342.

[pmid9072584-13] Morales A, Johnston B, Heaton JP, Lundie M (March 1997). "Testosterone supplementation for hypogonadal impotence: assessment of biochemical measures and therapeutic outcomes". J. Urol. 157 (3): 849–54. PMID 9072584.

[Elliott20082-14] Elliott, S (2008). "Erythropoiesis-stimulating agents and other methods to enhance oxygen transport". British Journal of Pharmacology. 154 (3): 529–541. doi:10.1038/bjp.2008.89. ISSN 0007-1188.

[NadeemGui2012-15] Nadeem, Omar; Gui, Jiang; Ornstein, Deborah L. (2012). "Prevalence of Venous Thromboembolism in Patients With Secondary Polycythemia". Clinical and Applied Thrombosis/Hemostasis. 19 (4): 363–366. doi:10.1177/1076029612460425. ISSN 1076-0296.

[pmid2297568-16] Da Silva JL, Lacombe C, Bruneval P, Casadevall N, Leporrier M, Camilleri JP, Bariety J, Tambourin P, Varet B (February 1990). "Tumor cells are the site of erythropoietin synthesis in human renal cancers associated with polycythemia". Blood. 75 (3): 577–82. PMID 2297568.

[17] McDonald J, Pyeritz RE. Hereditary Hemorrhagic Telangiectasia. 2000 Jun 26 [Updated 2017 Feb 2]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1351/

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@@ Line 3: / Line 3: @@
 {{CMG}} {{AE}}{{MJK}}; {{shyam}}
 ==Overview==
-Polycythemia vera must be differentiated from other myeloproliferative neoplasms, such as [[chronic myelogenous leukemia]], [[essential thrombocythemia]], and [[primary myelofibrosis]]. Polycythemia vera must also be differentiated from secondary polycythemia, which is usually due to chronic hypoxia. Each of these conditions have different etiologies, symptoms, laboratory abnormalities, physical exam findings, and treatments.
+Polycythemia vera must be differentiated from a variety of other conditions that might cause polycythemia. Polycythemia vera must also be differentiated from secondary polycythemia, which is usually due to chronic hypoxia. Each of these conditions has different etiologies, symptoms, laboratory abnormalities, physical exam findings, and treatments.
-==Differential Diagnosis==
+==Differential Diagnosis of Polycythemia Vera==
-Polycythemia vera must be differentiated from a variety of other conditions.<ref name="pmid25611051">{{cite journal| author=Tefferi A, Barbui T| title=Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management. | journal=Am J Hematol | year= 2015 | volume= 90 | issue= 2 | pages= 162-73 | pmid=25611051 | doi=10.1002/ajh.23895 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25611051  }} </ref><ref name="pmid16879015">{{cite journal| author=Sanchez S, Ewton A| title=Essential thrombocythemia: a review of diagnostic and pathologic features. | journal=Arch Pathol Lab Med | year= 2006 | volume= 130 | issue= 8 | pages= 1144-50 | pmid=16879015 | doi=10.1043/1543-2165(2006)130[1144:ET]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16879015  }} </ref><ref name="pmid24729196">{{cite journal| author=Jabbour E, Kantarjian H| title=Chronic myeloid leukemia: 2014 update on diagnosis, monitoring, and management. | journal=Am J Hematol | year= 2014 | volume= 89 | issue= 5 | pages= 547-56 | pmid=24729196 | doi=10.1002/ajh.23691 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24729196  }} </ref>
+Polycythemia vera must be differentiated from a variety of other conditions that might cause polycythemia.<ref name="pmid25611051">{{cite journal| author=Tefferi A, Barbui T| title=Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management. | journal=Am J Hematol | year= 2015 | volume= 90 | issue= 2 | pages= 162-73 | pmid=25611051 | doi=10.1002/ajh.23895 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25611051  }} </ref><ref name="pmid16879015">{{cite journal| author=Sanchez S, Ewton A| title=Essential thrombocythemia: a review of diagnostic and pathologic features. | journal=Arch Pathol Lab Med | year= 2006 | volume= 130 | issue= 8 | pages= 1144-50 | pmid=16879015 | doi=10.1043/1543-2165(2006)130[1144:ET]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16879015  }} </ref><ref name="pmid24729196">{{cite journal| author=Jabbour E, Kantarjian H| title=Chronic myeloid leukemia: 2014 update on diagnosis, monitoring, and management. | journal=Am J Hematol | year= 2014 | volume= 89 | issue= 5 | pages= 547-56 | pmid=24729196 | doi=10.1002/ajh.23691 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24729196  }} </ref>
 {|
+! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
 ! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Disease
-! colspan="2" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mutation
 ! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Etiology
-! colspan="10" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Clinical manifestations
+! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mutation
+! colspan="14" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Clinical manifestations
 ! colspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Laboratory findings
-! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Treatment
+! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard disgnosis
 ! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
 |-
-! colspan="5" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptoms
+! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
-! colspan="5" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Signs
+! colspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptoms
+! colspan="7" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Signs
 ! colspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |CBC
-! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Erythropoietin level
+! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |EPO level
 ! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |PBS
 |-
-! style="background: #4479BA; color: #FFFFFF; text-align: center;" |JAK2 mutation
-! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CALR mutation
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fatigue
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Headache
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Shortness of breath
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Bleeding
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other symptoms
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Appearance
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |SaO2
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fever
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |BP
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Tenderness
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Splenomegaly
@@ Line 40: / Line 43: @@
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Plt
 |-
-! align="center" style="background:#DCDCDC;" |[[Polycythemia vera (PV)]]
+! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mutational causes
-| align="center" style="background:#F5F5F5;" |> 95%
+! align="center" style="background:#DCDCDC;" |[[Polycythemia vera]] (PV)<ref name="SteinOh2015">{{cite journal|last1=Stein|first1=Brady L.|last2=Oh|first2=Stephen T.|last3=Berenzon|first3=Dmitriy|last4=Hobbs|first4=Gabriela S.|last5=Kremyanskaya|first5=Marina|last6=Rampal|first6=Raajit K.|last7=Abboud|first7=Camille N.|last8=Adler|first8=Kenneth|last9=Heaney|first9=Mark L.|last10=Jabbour|first10=Elias J.|last11=Komrokji|first11=Rami S.|last12=Moliterno|first12=Alison R.|last13=Ritchie|first13=Ellen K.|last14=Rice|first14=Lawrence|last15=Mascarenhas|first15=John|last16=Hoffman|first16=Ronald|title=Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery ofJAK2 V617F|journal=Journal of Clinical Oncology|volume=33|issue=33|year=2015|pages=3953–3960|issn=0732-183X|doi=10.1200/JCO.2015.61.6474}}</ref>
-| align="center" style="background:#F5F5F5;" |
+| align="left" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |Autonomous erythrocyte production
+* Autonomous erythrocyte production
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |JAK2 mutation > 95%
+| align="center" style="background:#F5F5F5;" | Mean age >60 years old
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | ±
 | align="left" style="background:#F5F5F5;" |
 * [[Erythromelagia]]
+* [[Chest pain]]
 | align="left" style="background:#F5F5F5;" |
 * [[Stroke]]-like symptoms
-| align="center" style="background:#F5F5F5;" |[[Ruddy face]]
+* [[Pruritis]]
-| align="center" style="background:#F5F5F5;" | -
+* Dizziness
-| align="center" style="background:#F5F5F5;" | -
+* Visual disturbance
+| align="center" style="background:#F5F5F5;" |Facial plethora
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | ↑
+| align="left" style="background:#F5F5F5;" |
+* Painful erythema
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |NA
+| align="left" style="background:#F5F5F5;" |
+* [[Lymphadenopathy]]
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑ RBC mass
 | align="center" style="background:#F5F5F5;" |Nl to ↑
 | align="center" style="background:#F5F5F5;" |Nl to ↑
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |
 | align="left" style="background:#F5F5F5;" |
-* [[Aspirin]]
+* Elevated normochromic, normocytic [[Red blood cell|RBCs]]
-* [[Ruxolitinib]]
+* [[Thrombocytosis]]
-* [[Hydroxyurea]]
+* Rarely immature cells
-* [[Phlebotomy]]
+* Leukoerythroblastic picture
-* [[Interferon-alpha]]
+| align="center" style="background:#F5F5F5;" |WHO criteria for PV
 | align="left" style="background:#F5F5F5;" |
 * [[Stroke]]
@@ Line 74: / Line 87: @@
 * [[Acute leukemia]]
 |-
-! align="center" style="background:#DCDCDC;" |[[Essential thrombocythemia]] (ET)
+! align="center" style="background:#DCDCDC;" |Chuvash polycythemia<ref name="ZhouKnoche2016">{{cite journal|last1=Zhou|first1=Amy W.|last2=Knoche|first2=Eric M.|last3=Engle|first3=Elizabeth K.|last4=Ban-Hoefen|first4=Makiko|last5=Kaiwar|first5=Charu|last6=Oh|first6=Stephen T.|title=Clinical Improvement with JAK2 Inhibition in Chuvash Polycythemia|journal=New England Journal of Medicine|volume=375|issue=5|year=2016|pages=494–496|issn=0028-4793|doi=10.1056/NEJMc1600337}}</ref>
-| align="center" style="background:#F5F5F5;" |50%
+| align="center" style="background:#F5F5F5;" | Hypoxia-sensing disorder
-| align="center" style="background:#F5F5F5;" | +/-
+| align="center" style="background:#F5F5F5;" | VHL mutation
-| align="left" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | Russia, Italy
-* Clonal proliferation of megakaryocytes
+<40 years old
-* Excess [[platelet]] production
+| align="center" style="background:#F5F5F5;" |  +
+| align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ±
-| align="center" style="background:#F5F5F5;" | + (paradoxical)
+| align="left" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |
+* [[Arthralgia]]
 | align="left" style="background:#F5F5F5;" |
-* Stroke-like symptoms
+* [[Pruritis]]
-| align="center" style="background:#F5F5F5;" |
+* Dizziness
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | Facial plethora
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ↓
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | ↑
+| align="left" style="background:#F5F5F5;" |
+* Painful erythema
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |[[Bruises]]
+| align="left" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |
+* [[Lymphadenopathy]]
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ↑
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ↑
-| align="center" style="background:#F5F5F5;" |↑ (dysfunctional platelets)
+| align="center" style="background:#F5F5F5;" | Nl
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | Nl
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ↑
 | align="left" style="background:#F5F5F5;" |
-* [[Aspirin]]
+* Elevated normochromic, normocytic [[Red blood cell|RBCs]]
-* [[Hydroyurea]]
+| align="center" style="background:#F5F5F5;" | Molecular genetic testing
-* [[Anagrelide]]
+| align="left" style="background:#F5F5F5;" |
-| align="left" style="background:#F5F5F5;" |
+* Multiple thrombotic events
-* Acquired [[von Willebrand disease]]
-* [[Myelofibrosis]]
-* [[Acute leukemia]]
 |-
-! align="center" style="background:#DCDCDC;" |[[Chronic myeloid leukemia]] (CML)
+! align="center" style="background:#DCDCDC;" |Hereditary [[methemoglobinemia]]<ref name="pmid12897322">{{cite journal |vauthors=Da-Silva SS, Sajan IS, Underwood JP |title=Congenital methemoglobinemia: a rare cause of cyanosis in the newborn--a case report |journal=Pediatrics |volume=112 |issue=2 |pages=e158–61 |date=August 2003 |pmid=12897322 |doi= |url=}}</ref>
+| align="left" style="background:#F5F5F5;" |
+* Cytochrome b5 reductase deficiency
+* Hemoglobin M disease
+| align="center" style="background:#F5F5F5;" | Mutations in globin gene
+| align="center" style="background:#F5F5F5;" | Infants
+| align="center" style="background:#F5F5F5;" | ±
+| align="center" style="background:#F5F5F5;" | ±
+| align="center" style="background:#F5F5F5;" | ±
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | [[Cyanosis]]
+| align="center" style="background:#F5F5F5;" | Inaccurately Nl
 | align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="left" style="background:#F5F5F5;" |
+* Altered mental status
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | NA
+| align="center" style="background:#F5F5F5;" | RBC enzyme activity + DNA analysis
+| align="left" style="background:#F5F5F5;" |
+* Usually asymptomatic
+|-
+! align="center" style="background:#DCDCDC;" |Primary familial and congenital polycythemia<ref>Bento C, McMullin MF, Percy M, et al. Primary Familial and Congenital Polycythemia. 2016 Nov 10. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK395975/</ref>
+| align="center" style="background:#F5F5F5;" | Autosomal dominant inheritance
+| align="center" style="background:#F5F5F5;" | EPOR mutation
+| align="center" style="background:#F5F5F5;" | Very rare
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | ±
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | Facial plethora
+| align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" | -
-| align="left" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | Nl to ↑
-* Reciprocal translocation of chromosomes 9 and 22
+| align="center" style="background:#F5F5F5;" | −
-* Production of BCR-Abl kinase
+| align="center" style="background:#F5F5F5;" | −
+| align="left" style="background:#F5F5F5;" |
+* Altered mental status
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl to ↓
+| align="center" style="background:#F5F5F5;" | NA
+| align="center" style="background:#F5F5F5;" | Isolated erythrocytosis + genetic testing
+| align="left" style="background:#F5F5F5;" |
+* Mild manifestations of hyperviscosity
+|-
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Disease
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Etiology
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mutation
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fatigue
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Headache
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Shortness of breath
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Bleeding
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other symptoms
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Appearance
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |SaO2
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fever
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |BP
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Tenderness
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Splenomegaly
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other signs
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hb
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |RBC
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |WBC
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Plt
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |EPO level
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |PBS
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
+|-
+! rowspan="5" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Iatrogenic causes
+! align="center" style="background:#DCDCDC;" |[[Smoking]]<ref name="Hasselbalch2015">{{cite journal|last1=Hasselbalch|first1=Hans Carl|title=Smoking as a contributing factor for development of polycythemia vera and related neoplasms|journal=Leukemia Research|volume=39|issue=11|year=2015|pages=1137–1145|issn=01452126|doi=10.1016/j.leukres.2015.09.002}}</ref><ref name="MalenicaPrnjavorac2017">{{cite journal|last1=Malenica|first1=Maja|last2=Prnjavorac|first2=Besim|last3=Bego|first3=Tamer|last4=Dujic|first4=Tanja|last5=Semiz|first5=Sabina|last6=Skrbo|first6=Selma|last7=Gusic|first7=Amar|last8=Hadzic|first8=Ajla|last9=Causevic|first9=Adlija|title=Effect of Cigarette Smoking on Haematological Parameters in Healthy Population|journal=Medical Archives|volume=71|issue=2|year=2017|pages=132|issn=0350-199X|doi=10.5455/medarh.2017.71.132-136}}</ref>
+| align="left" style="background:#F5F5F5;" |
+* Reduced plasma volume
+* Accelerated [[erythropoiesis]]
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | Any
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
 | align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | [[Cyanosis]]
+| align="center" style="background:#F5F5F5;" | ↓
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" |Nl
+| align="center" style="background:#F5F5F5;" | Nl to ↑
+| align="center" style="background:#F5F5F5;" | Nl to ↑
+| align="center" style="background:#F5F5F5;" | Nl to ↓
+| align="center" style="background:#F5F5F5;" | NA
+| align="center" style="background:#F5F5F5;" | Clinical manifestation
+| align="center" style="background:#F5F5F5;" | NA
+|-
+! align="center" style="background:#DCDCDC;" |[[Carbon monoxide poisoning|Chronic exposure to carbon monoxide]]<ref name="WuJuurlink2014">{{cite journal|last1=Wu|first1=P. E.|last2=Juurlink|first2=D. N.|title=Carbon monoxide poisoning|journal=Canadian Medical Association Journal|volume=186|issue=8|year=2014|pages=611–611|issn=0820-3946|doi=10.1503/cmaj.130972}}</ref>
+| align="left" style="background:#F5F5F5;" |
+* Occupational exposure
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | Miners, fire fighters
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |[[Abdominal pain]]
-| align="center" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | −
-| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | −
-| align="center" style="background:#F5F5F5;" |
+| align="left" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |↑
+* Dizziness
-| align="center" style="background:#F5F5F5;" |↑
+* Altered cognition
-| align="center" style="background:#F5F5F5;" |↑
+| align="center" style="background:#F5F5F5;" | [[Cyanosis]], flushed cheeks
-| align="center" style="background:#F5F5F5;" |↑
+| align="center" style="background:#F5F5F5;" |Nl
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | −
-| align="center" style="background:#F5F5F5;" |Elevated metamyelocytes and other [[white blood cells]] at various stages of maturation
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl to ↓
+| align="center" style="background:#F5F5F5;" | NA
+| align="center" style="background:#F5F5F5;" | Blood level of [[carboxyhemoglobin]]
 | align="left" style="background:#F5F5F5;" |
-* [[Imatinib]]
+* Concomitant [[cyanide]] exposure
-* [[Dasatinib]]
+|-
-* [[Bosutinib]]
+! align="center" style="background:#DCDCDC;" |[[Diuretic|Diuretics]]<ref name="pmid3282731">{{cite journal |vauthors=Pollak R, Maddux MS, Cohan J, Jacobsson PK, Mozes MF |title=Erythrocythemia following renal transplantation: influence of diuretic therapy |journal=Clin. Nephrol. |volume=29 |issue=3 |pages=119–23 |date=March 1988 |pmid=3282731 |doi= |url=}}</ref>
-* [[Nilotinib]]
-* [[Ponatinib]] for T315I kinase domain mutation
-* [[Interferon-alpha]]
 | align="left" style="background:#F5F5F5;" |
-* Can appear similar to [[leukemoid reaction]]
+* Reduced plasma volume
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" |Any
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="left" style="background:#F5F5F5;" |
+* Dizziness
+| align="center" style="background:#F5F5F5;" |Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | Nl to ↓
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" |Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl to ↓
+| align="center" style="background:#F5F5F5;" | NA
+| align="center" style="background:#F5F5F5;" | Clinical manifestation
+| align="center" style="background:#F5F5F5;" | NA
 |-
-! align="center" style="background:#DCDCDC;" |[[Primary myelofibrosis]] (PMF)
+! align="center" style="background:#DCDCDC;" |Use of [[Androgen|androgens]] or [[Anabolic steroid|anabolic steroids]]<ref name="pmid1942342">{{cite journal |vauthors=Krauss DJ, Taub HA, Lantinga LJ, Dunsky MH, Kelly CM |title=Risks of blood volume changes in hypogonadal men treated with testosterone enanthate for erectile impotence |journal=J. Urol. |volume=146 |issue=6 |pages=1566–70 |date=December 1991 |pmid=1942342 |doi= |url=}}</ref><ref name="pmid9072584">{{cite journal |vauthors=Morales A, Johnston B, Heaton JP, Lundie M |title=Testosterone supplementation for hypogonadal impotence: assessment of biochemical measures and therapeutic outcomes |journal=J. Urol. |volume=157 |issue=3 |pages=849–54 |date=March 1997 |pmid=9072584 |doi= |url=}}</ref>
-| align="center" style="background:#F5F5F5;" |
+| align="left" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |
+* Reduced plasma volume
-| align="left" style="background:#F5F5F5;" |
+* Accelerated [[erythropoiesis]]
-* Clonal disorder of [[megakaryocytes]]
+| align="center" style="background:#F5F5F5;" | -
-* Excess production [[TGF-beta]] and [[collagen]]
+| align="center" style="background:#F5F5F5;" | Athletes
+| align="center" style="background:#F5F5F5;" | -
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" |[[Abdominal pain]]
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | [[Acne vulgaris|Acne]] and [[hirsutism]]
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" |Nl to ↑
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
 | align="left" style="background:#F5F5F5;" |
-* Early satiety
+* Gynecomastia
-| align="center" style="background:#F5F5F5;" |Pallor
+| align="center" style="background:#F5F5F5;" | ↑
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ↑
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | Nl
-| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | Nl
-| align="left" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ↓
-* [[Hepatomegaly]]
+| align="center" style="background:#F5F5F5;" |NA
-* [[Bruising]]
+| align="center" style="background:#F5F5F5;" | Clinical manifestation
-| align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |
-| align="left" style="background:#F5F5F5;" |
-* [[Hydroxyurea]]
-* [[Stem cell transplant]]
-* [[Ruxolitinib]]
 | align="left" style="background:#F5F5F5;" |
-* Variable risk for development of [[acute leukemia]]
+* [[Hypogonadism]]
+* [[Cardiomegaly|Cardiac hypertrophy]]
 |-
-! align="center" style="background:#DCDCDC;" |Secondary polycythemia due to chronic hypoxia
+! align="center" style="background:#DCDCDC;" |Self-injection of [[erythropoietin]]<ref name="Elliott20082">{{cite journal|last1=Elliott|first1=S|title=Erythropoiesis-stimulating agents and other methods to enhance oxygen transport|journal=British Journal of Pharmacology|volume=154|issue=3|year=2008|pages=529–541|issn=00071188|doi=10.1038/bjp.2008.89}}</ref>
+| align="left" style="background:#F5F5F5;" |
+* Reduced plasma volume
+* Accelerated [[erythropoiesis]]
 | align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | Athletes
 | align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | Muscular
+| align="center" style="background:#F5F5F5;" | ↓
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" |↑
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | ↑
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" |↑
+| align="center" style="background:#F5F5F5;" |NA
+| align="center" style="background:#F5F5F5;" |[[Erythropoietin]] level
 | align="left" style="background:#F5F5F5;" |
-* [[Congestive heart failure]] (CHF)
+* [[ST elevation myocardial infarction|MI]]
-* [[Interstitial lung disease]] (ILD)
+* [[Stroke]]
-* [[Obstructive sleep apnea]] (OSA)
+* [[Thrombosis|Thromboembolic disease]]
-* Smoking
+* antibody-mediated anemia
+|-
+! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Secondary causes
+! align="center" style="background:#DCDCDC;" |Secondary polycythemia due to [[hypoxemia]]<ref name="NadeemGui2012">{{cite journal|last1=Nadeem|first1=Omar|last2=Gui|first2=Jiang|last3=Ornstein|first3=Deborah L.|title=Prevalence of Venous Thromboembolism in Patients With Secondary Polycythemia|journal=Clinical and Applied Thrombosis/Hemostasis|volume=19|issue=4|year=2012|pages=363–366|issn=1076-0296|doi=10.1177/1076029612460425}}</ref>
+| align="left" style="background:#F5F5F5;" |
+* Chronic lung disease
+* [[Right-to-left shunt|Right-to-left cardiac shunts]]
+* [[Sleep apnea]]
+* [[Altitude sickness|High altitude]]
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | Depends on etiology
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" |  +
+| align="center" style="background:#F5F5F5;" | −
 | align="center" style="background:#F5F5F5;" |Depends on etiology
 | align="left" style="background:#F5F5F5;" |
 * [[Shortness of breath]]
 | align="center" style="background:#F5F5F5;" |[[Cyanosis]]
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |↓
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |−
-| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" |Nl to ↑
+| align="center" style="background:#F5F5F5;" |−
+| align="center" style="background:#F5F5F5;" |−
 | align="left" style="background:#F5F5F5;" |
 * [[Crackles]]
@@ Line 197: / Line 388: @@
 | align="center" style="background:#F5F5F5;" |Nl
 | align="center" style="background:#F5F5F5;" |↑
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |NA
-| align="left" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |Clinical manifestation + imaging
-* Treatment of underlying cause
 | align="left" style="background:#F5F5F5;" |
 * Variable manifestations given the diverse etiologies
 |-
-! align="center" style="background:#DCDCDC;" |Secondary polycythemia due to [[erythropoietin]] producing tumor
+! align="center" style="background:#DCDCDC;" |Secondary polycythemia due to [[erythropoietin]] producing tumor<ref name="pmid2297568">{{cite journal |vauthors=Da Silva JL, Lacombe C, Bruneval P, Casadevall N, Leporrier M, Camilleri JP, Bariety J, Tambourin P, Varet B |title=Tumor cells are the site of erythropoietin synthesis in human renal cancers associated with polycythemia |journal=Blood |volume=75 |issue=3 |pages=577–82 |date=February 1990 |pmid=2297568 |doi= |url=}}</ref>
-| align="center" style="background:#F5F5F5;" | -
-| align="center" style="background:#F5F5F5;" | -
 | align="left" style="background:#F5F5F5;" |
-* Renal cell carcinoma
+*[[Renal cell carcinoma]]
-* Hepatocellular carcinoma
+*[[Hepatocellular carcinoma]]
+*Cerebellar [[hemangioblastoma]]
+*[[Pheochromocytoma]]
+*[[Leiomyoma|Uterine fibroids]]
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | Depends on etiology
 | align="center" style="background:#F5F5F5;" | +
-| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | −
-| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | −
+| align="center" style="background:#F5F5F5;" | −
 | align="center" style="background:#F5F5F5;" |Depends on etiology
 | align="center" style="background:#F5F5F5;" |Depends on etiology
 | align="center" style="background:#F5F5F5;" |Chronically ill
-| align="center" style="background:#F5F5F5;" | +/-
+| align="center" style="background:#F5F5F5;" | Nl
-| align="center" style="background:#F5F5F5;" | +/-
+| align="center" style="background:#F5F5F5;" | ±
-| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | Nl to ↑
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" | ±
+| align="center" style="background:#F5F5F5;" | ±
+| align="left" style="background:#F5F5F5;" |
 * [[Hepatomegaly]]
 * [[Bruising]]
@@ Line 226: / Line 422: @@
 | align="center" style="background:#F5F5F5;" |↑
 | align="center" style="background:#F5F5F5;" |↑
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |NA
-| align="center" style="background:#F5F5F5;" |
+| align="center" style="background:#F5F5F5;" |Clinical manifestation + [[biopsy]]
-* Surgery
+| align="left" style="background:#F5F5F5;" |
-* Chemotherapy
-| align="center" style="background:#F5F5F5;" |
 * Variable manifestations given the diverse etiologies
 |-
+! align="center" style="background:#DCDCDC;" |[[Hereditary hemorrhagic telangiectasia]]<ref>McDonald J, Pyeritz RE. Hereditary Hemorrhagic Telangiectasia. 2000 Jun 26 [Updated 2017 Feb 2]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1351/</ref>
+| align="left" style="background:#F5F5F5;" |
+* [[Pulmonary arteriovenous malformation|Pulmonary arteriovenous malformations]]
+| align="center" style="background:#F5F5F5;" |ACVRL1, ENG, GDF2, SMAD4 mutation
+| align="center" style="background:#F5F5F5;" |Children
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | +
+| align="center" style="background:#F5F5F5;" | [[Gastrointestinal bleeding|GI bleeding]]
+| align="center" style="background:#F5F5F5;" | -
+| align="left" style="background:#F5F5F5;" |
+* [[Epistaxis]]
+* [[Hemoptysis]]
+| align="center" style="background:#F5F5F5;" |Multiple [[telangiectasia]] on face, extremities, and body
+| align="center" style="background:#F5F5F5;" | Nl to ↓
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | -
+| align="center" style="background:#F5F5F5;" | ↑/↓
+| align="center" style="background:#F5F5F5;" | ↑/↓
+| align="center" style="background:#F5F5F5;" |Nl
+| align="center" style="background:#F5F5F5;" | Nl
+| align="center" style="background:#F5F5F5;" | ↑/↓
+| align="center" style="background:#F5F5F5;" |NA
+| align="center" style="background:#F5F5F5;" |Clinical criteria + genomic testing
+| align="left" style="background:#F5F5F5;" |
+* [[Dominance relationship|Autosomal dominant]]
+* [[Pulmonary hypertension]]
+* [[Cerebral arteriovenous malformation|Cerebral AVMs]]
+|-
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Disease
-! style="background: #4479BA; color: #FFFFFF; text-align: center;" |JAK2 mutation
-! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CALR mutation
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Etiology
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mutation
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fatigue
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Headache
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Shortness of breath
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Bleeding
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other symptoms
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Appearance
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |SaO2
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Fever
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |BP
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Tenderness
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Splenomegaly
@@ Line 251: / Line 481: @@
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |WBC
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Plt
-! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Erythropoietin level
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |EPO level
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |PBS
-! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Treatment
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
 |}
 ==References==
-{{Reflist|2}}
+{{reflist|2}}
+[[Category:Medicine]]
+[[Category:Hematology]]
+[[Category:Up-To-Date]]
+[[Category:Primary care]]

Polycythemia vera differential diagnosis: Difference between revisions

Revision as of 13:56, 24 August 2018

Overview

Differential Diagnosis of Polycythemia Vera

References

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