Polycythemia vera differential diagnosis: Difference between revisions
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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.<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> | ||
*Acute leukemia | *[[Acute leukemia]] | ||
*Essential thrombocythemia | *[[Essential thrombocythemia]] | ||
* | *[[Chronic myeloid leukemia]] | ||
*[[Primary myelofibrosis]] | |||
*Primary myelofibrosis | |||
*Secondary polycythemia | *Secondary polycythemia | ||
{| | {| | ||
| Line 25: | Line 23: | ||
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other associated abnormalities | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other associated abnormalities | ||
|- | |- | ||
! | ! style="background:#DCDCDC;" align="center" |[[Polycythemia vera (PV)]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[JAK2]] mutation (V617F or exon 12 mutation) in more than 95% of cases | * [[JAK2]] mutation (V617F or exon 12 mutation) in more than 95% of cases | ||
* Autonomous [[Red blood cell|erythrocyte]] production | * Autonomous [[Red blood cell|erythrocyte]] production | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Erythromelagia]] | * [[Erythromelagia]] | ||
* [[Headache]] | * [[Headache]] | ||
* [[Stroke]]-like symptoms | * [[Stroke]]-like symptoms | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Ruddy face]] | * [[Ruddy face]] | ||
* [[Splenomegaly]] | * [[Splenomegaly]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* ↑ [[Hemoglobin]] | * ↑ [[Hemoglobin]] | ||
* ↑ [[Red blood cell|RBC]] mass | * ↑ [[Red blood cell|RBC]] mass | ||
| Line 42: | Line 40: | ||
* ↑ [[Platelet]] | * ↑ [[Platelet]] | ||
* Panmyelosis | * Panmyelosis | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Aspirin]] | * [[Aspirin]] | ||
* [[Ruxolitinib]] | * [[Ruxolitinib]] | ||
| Line 48: | Line 46: | ||
* [[Phlebotomy]] | * [[Phlebotomy]] | ||
* [[Interferon-alpha]] | * [[Interferon-alpha]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Extramedullary hematopoiesis]] | * [[Extramedullary hematopoiesis]] | ||
* [[Stroke]] | * [[Stroke]] | ||
| Line 54: | Line 52: | ||
* Can progress to post-PV [[myelofibrosis]] and eventually [[acute leukemia]] | * Can progress to post-PV [[myelofibrosis]] and eventually [[acute leukemia]] | ||
|- | |- | ||
! | ! style="background:#DCDCDC;" align="center" |[[Essential thrombocythemia]] (ET) | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Clonal proliferation of [[Megakaryocyte|megakaryocytes]] | * Clonal proliferation of [[Megakaryocyte|megakaryocytes]] | ||
* Excess [[platelet]] production | * Excess [[platelet]] production | ||
* Caused by [[Janus kinase|JAK2]] mutation in 50% of cases | * Caused by [[Janus kinase|JAK2]] mutation in 50% of cases | ||
* Caused by CALR mutation in some cases | * Caused by CALR mutation in some cases | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Stroke]]-like symptoms | * [[Stroke]]-like symptoms | ||
* [[Fatigue]] | * [[Fatigue]] | ||
* [[Bleeding]] due to dysfunctional platelets and acquired [[von Willebrand disease]] | * [[Bleeding]] due to dysfunctional platelets and acquired [[von Willebrand disease]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Splenomegaly]] | * [[Splenomegaly]] | ||
* [[Bruise|Bruising]] | * [[Bruise|Bruising]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* ↑ [[Platelet]] | * ↑ [[Platelet]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Aspirin]] | * [[Aspirin]] | ||
* [[Hydroxyurea]] | * [[Hydroxyurea]] | ||
* [[Anagrelide]] | * [[Anagrelide]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Paradoxical [[bleeding]] | * Paradoxical [[bleeding]] | ||
* Can progress to post-ET [[myelofibrosis]] | * Can progress to post-ET [[myelofibrosis]] | ||
* Can develop into [[acute leukemia]] | * Can develop into [[acute leukemia]] | ||
|- | |- | ||
! | ! style="background:#DCDCDC;" align="center" |[[Chronic myeloid leukemia]] (CML) | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Reciprocal translocation of chromosomes 9 and 22 | * Reciprocal translocation of chromosomes 9 and 22 | ||
* Production of [[BCR/ABL|BCR-Abl kinase]], which drives cell proliferation | * Production of [[BCR/ABL|BCR-Abl kinase]], which drives cell proliferation | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Abdominal pain]] | * [[Abdominal pain]] | ||
* [[Infection|Infections]] | * [[Infection|Infections]] | ||
* [[Fever]] | * [[Fever]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Splenomegaly]] | * [[Splenomegaly]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* ↑ [[Red blood cell|RBC]] mass | * ↑ [[Red blood cell|RBC]] mass | ||
* ↑ [[White blood cells|WBC]] | * ↑ [[White blood cells|WBC]] | ||
* ↑ [[Platelet]] | * ↑ [[Platelet]] | ||
* ↑ Proportion of metamyelocytes and other [[white blood cells]] at various stages of maturation | * ↑ Proportion of metamyelocytes and other [[white blood cells]] at various stages of maturation | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Imatinib]] | * [[Imatinib]] | ||
* [[Dasatinib]] | * [[Dasatinib]] | ||
| Line 100: | Line 98: | ||
* [[Ponatinib]] for T315I kinase domain mutation | * [[Ponatinib]] for T315I kinase domain mutation | ||
* [[Interferon-alpha]] | * [[Interferon-alpha]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Can appear similar to [[leukemoid reaction]] | * Can appear similar to [[leukemoid reaction]] | ||
|- | |- | ||
! | ! style="background:#DCDCDC;" align="center" |[[Primary myelofibrosis]] (PMF) | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Clonal disorder of [[megakaryocytes]] | * Clonal disorder of [[megakaryocytes]] | ||
* Excess production [[TGF-beta]] and [[collagen]] | * Excess production [[TGF-beta]] and [[collagen]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Fatigue]] | * [[Fatigue]] | ||
* [[Abdominal pain]] | * [[Abdominal pain]] | ||
* Early satiety | * Early satiety | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Splenomegaly]] | * [[Splenomegaly]] | ||
* [[Hepatomegaly]] | * [[Hepatomegaly]] | ||
* [[Pallor]] | * [[Pallor]] | ||
* [[Bruising]] | * [[Bruising]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* ↓ [[Hemoglobin]] | * ↓ [[Hemoglobin]] | ||
* ↓ [[White blood cells|WBC]] | * ↓ [[White blood cells|WBC]] | ||
* ↓ [[Platelet]] | * ↓ [[Platelet]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Hydroxyurea]] | * [[Hydroxyurea]] | ||
* [[Stem cell transplant]] | * [[Stem cell transplant]] | ||
* [[Ruxolitinib]] | * [[Ruxolitinib]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Variable risk for development of [[acute leukemia]] | * Variable risk for development of [[acute leukemia]] | ||
|- | |- | ||
! | ! style="background:#DCDCDC;" align="center" |Secondary polycythemia | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Congestive heart failure]] | * [[Congestive heart failure]] | ||
* [[Interstitial lung disease]] | * [[Interstitial lung disease]] | ||
| Line 136: | Line 134: | ||
* Ectopic [[erythropoietin]] production from tumor ([[renal cell carcinoma]] or [[hepatocellular carcinoma]]) | * Ectopic [[erythropoietin]] production from tumor ([[renal cell carcinoma]] or [[hepatocellular carcinoma]]) | ||
* Chronic [[hypoxia]] from a variety of other causes | * Chronic [[hypoxia]] from a variety of other causes | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Headache]] | * [[Headache]] | ||
* [[Fatigue]] | * [[Fatigue]] | ||
* [[Shortness of breath]] | * [[Shortness of breath]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* [[Crackles]] | * [[Crackles]] | ||
* [[Cyanosis]] | * [[Cyanosis]] | ||
* [[Tachypnea]] | * [[Tachypnea]] | ||
* [[Tachycardia]] | * [[Tachycardia]] | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* ↑ [[Hemoglobin]] | * ↑ [[Hemoglobin]] | ||
* ↑ [[Erythropoietin]] level | * ↑ [[Erythropoietin]] level | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Treatment of underlying cause of [[hypoxia]] | * Treatment of underlying cause of [[hypoxia]] | ||
* Supplemental oxygen | * Supplemental oxygen | ||
| Line 156: | Line 154: | ||
* [[Smoking cessation]] or nicotine replacement | * [[Smoking cessation]] or nicotine replacement | ||
* Surgical removal of tumors | * Surgical removal of tumors | ||
| | | style="background:#F5F5F5;" align="left" | | ||
* Variable manifestations given the diverse etiologies | * Variable manifestations given the diverse etiologies | ||
|- | |- | ||
Revision as of 23:22, 22 January 2019
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Polycythemia vera Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Polycythemia vera differential diagnosis On the Web |
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American Roentgen Ray Society Images of Polycythemia vera differential diagnosis |
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Risk calculators and risk factors for Polycythemia vera differential diagnosis |
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 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.
Differential Diagnosis
Polycythemia vera must be differentiated from a variety of other conditions.[1][2][3]
- Acute leukemia
- Essential thrombocythemia
- Chronic myeloid leukemia
- Primary myelofibrosis
- Secondary polycythemia
| Disease | Etiology | Symptoms | Signs | Laboratory abnormalities | Treatment | Other associated abnormalities |
|---|---|---|---|---|---|---|
| Polycythemia vera (PV) |
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| Essential thrombocythemia (ET) |
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| Chronic myeloid leukemia (CML) |
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| Primary myelofibrosis (PMF) |
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| Secondary polycythemia |
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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.