Polycythemia vera differential diagnosis: Difference between revisions

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Revision as of 17:02, 20 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 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]

Disease	Etiology	Symptoms	Laboratory abnormalities	Physical examination	Treatment	Other associated abnormalities
Polycythemia vera (PV)	JAK2 mutation (V617F or exon 12 mutation) in more than 95% of cases Autonomous erythrocyte production	Erythromelagia Headache Stroke-like symptoms	Elevated hemoglobin Elevated red blood cell mass Elevated white blood cell count and platelet count in some cases (panmyelosis)	Ruddy face Splenomegaly	Aspirin Ruxolitinib Hydroxyurea Phlebotomy Interferon-alpha	Extramedullary hematopoiesis Stroke Venous thrombosis Can progress to post-PV myelofibrosis and eventually acute leukemia
Essential thrombocythemia (ET)	Clonal proliferation of megakaryocytes Excess platelet production Caused by JAK2 mutation in 50% of cases Caused by CALR mutation in some cases	Stroke-like symptoms Fatigue Bleeding due to dysfunctional platelets and acquired von Willebrand disease	Elevated total platelet count	Splenomegaly Bruising	Aspirin Hydroyurea Anagrelide	Paradoxical bleeding can occur when platelet count is above 1,000,000 cells/microliter due to acquired von Willebrand disease Can progress to post-ET myelofibrosis Can develop into acute leukemia
Chronic myeloid leukemia (CML)	Reciprocal translocation of chromosomes 9 and 22 Production of BCR-Abl kinase, which drives cell proliferation	Abdominal pain Infections Fever	Elevated total white blood cell count Elevated red blood cell count and platelet count occasionally Increased proportion of metamyelocytes and other white blood cells at various stages of maturation	Splenomegaly	Imatinib Dasatinib Bosutinib Nilotinib Ponatinib for T315I kinase domain mutation Interferon-alpha	Can appear similar to leukemoid reaction
Primary myelofibrosis (PMF)	Clonal disorder of megakaryocytes Excess production TGF-beta and collagen	Fatigue Abdominal pain Early satiety	Decreased hemoglobin (anemia) Decreased platelet count (thrombocytopenia) Decreased white blood cell count (leukopenia)	Splenomegaly Hepatomegaly Pallor Bruising	Hydroxyurea Stem cell transplant Ruxolitinib	Variable risk for development of acute leukemia
Secondary polycythemia	Congestive heart failure (CHF) Interstitial lung disease (ILD) Obstructive sleep apnea (OSA) Smoking High altitude living Ectopic erythropoietin production from tumor (renal cell carcinoma or hepatocellular carcinoma) Chronic hypoxia from a variety of other causes	Headache Fatigue Shortness of breath	Elevated hemoglobin Increased erythropoietin level	Crackles Cyanosis Tachypnea Tachycardia	Treatment of underlying cause of hypoxia Supplemental oxygen Diuresis for CHF Steroids for ILD Continuous positive airway pressure for OSA Smoking cessation or nicotine replacement Surgical removal of tumors	Variable manifestations given the diverse etiologies

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.

[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.

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[2]

[3]

@@ Line 6: / Line 6: @@
 ==Differential Diagnosis==
-Polycythemia vera must be differentiated from a variety of other conditions.
+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>
-{| class="wikitable"
+{|
-!Characteristic/Parameter
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Disease
-!Etiology
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Etiology
-!Symptoms
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptoms
-!Laboratory abnormalities
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Laboratory abnormalities
-!Physical examination
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
-!Treatment
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Treatment
-!Other associated abnormalities
+! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other associated abnormalities
 |-
-||[[Polycythemia vera (PV)]]
+! align="center" style="background:#DCDCDC;" |[[Polycythemia vera (PV)]]
 |
 * [[JAK2]] mutation (V617F or exon 12 mutation) in more than 95% of cases
@@ Line 44: / Line 44: @@
 * Can progress to post-PV [[myelofibrosis]] and eventually [[acute leukemia]]
 |-
-||[[Essential thrombocythemia]] (ET)
+! align="center" style="background:#DCDCDC;" |[[Essential thrombocythemia]] (ET)
 |
 * Clonal proliferation of megakaryocytes
@@ Line 68: / Line 68: @@
 * Can develop into acute leukemia
 |-
-||[[Chronic myeloid leukemia]] (CML)
+! align="center" style="background:#DCDCDC;" |[[Chronic myeloid leukemia]] (CML)
 |
 * Reciprocal translocation of chromosomes 9 and 22
@@ Line 92: / Line 92: @@
 * Can appear similar to [[leukemoid reaction]]
 |-
-|[[Primary myelofibrosis]] (PMF)
+! align="center" style="background:#DCDCDC;" |[[Primary myelofibrosis]] (PMF)
 |
 * Clonal disorder of [[megakaryocytes]]
@@ Line 116: / Line 116: @@
 * Variable risk for development of [[acute leukemia]]
 |-
-||Secondary polycythemia
+! align="center" style="background:#DCDCDC;" |Secondary polycythemia
 |
 * [[Congestive heart failure]] (CHF)
@@ Line 150: / Line 150: @@
 |}
-:<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>
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Polycythemia vera differential diagnosis: Difference between revisions

Revision as of 17:02, 20 August 2018

Overview

Differential Diagnosis

References

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