Polycythemia vera differential diagnosis: Difference between revisions

Jump to navigation Jump to search
No edit summary
No edit summary
Line 19: Line 19:
||[[Polycythemia vera (PV)]]   
||[[Polycythemia vera (PV)]]   
|
|
* 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 erythrocyte production
* Autonomous erythrocyte production
|
|
* Erythromelagia
* [[Erythromelagia]]
* Headache
* [[Headache]]
* Stroke-like symptoms
* [[Stroke]]-like symptoms
|
|
* Elevated [[hemoglobin]]
* Elevated [[hemoglobin]]
Line 33: Line 33:
* [[Splenomegaly]]
* [[Splenomegaly]]
|
|
* Aspirin
* [[Aspirin]]
* Ruxolitinib
* [[Ruxolitinib]]
* Hydroxyurea
* [[Hydroxyurea]]
* Phlebotomy
* [[Phlebotomy]]
* Interferon-alpha
* [[Interferon-alpha]]
|
|
* [[Extramedullary hematopoiesis]]
* [[Extramedullary hematopoiesis]]
* [[Stroke]]
* [[Stroke]]
* [[Venous thrombosis]]
* [[Venous thrombosis]]
* Can progress to post-PV myelofibrosis and eventually acute leukemia
* Can progress to post-PV [[myelofibrosis]] and eventually [[acute leukemia]]
|-
|-
||[[Essential thrombocythemia (ET)]]  
||[[Essential thrombocythemia]] (ET)   
|
|
* Clonal proliferation of megakaryocytes
* Clonal proliferation of megakaryocytes
Line 52: Line 52:
|
|
* 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]]
|
|
* Elevated total [[platelet]] count
* Elevated total [[platelet]] count
Line 60: Line 60:
* Bruising
* Bruising
|
|
* Aspirin
* [[Aspirin]]
* [[Hydroyurea]]
* [[Hydroyurea]]
* [[Anagrelide]]
* [[Anagrelide]]
Line 68: Line 68:
* Can develop into acute leukemia
* Can develop into acute leukemia
|-
|-
||[[Chronic myeloid leukemia (CML)]]  
||[[Chronic myeloid leukemia]] (CML)   
|
|
* Reciprocal translocation of chromosomes 9 and 22
* Reciprocal translocation of chromosomes 9 and 22
* Production of BCR-Abl kinase, which drives cell proliferation
* Production of BCR-Abl kinase, which drives cell proliferation
|
|
* Abdominal pain
* [[Abdominal pain]]
* Infections
* [[Infection|Infections]]
* Fever
* [[Fever]]
|
|
* Elevated total [[white blood cell count]]
* Elevated total [[white blood cell count]]
Line 81: Line 81:
* Increased proportion of metamyelocytes and other [[white blood cells]] at various stages of maturation
* Increased proportion of metamyelocytes and other [[white blood cells]] at various stages of maturation
|
|
* Splenomegaly
* [[Splenomegaly]]
|
|
* Imatinib
* [[Imatinib]]
* Dasatinib
* [[Dasatinib]]
* Bosutinib
* [[Bosutinib]]
* Nilotinib
* [[Nilotinib]]
* Ponatinib for T315I kinase domain mutation
* [[Ponatinib]] for T315I kinase domain mutation
* Interferon-alpha
* [[Interferon-alpha]]
|
|
* Can appear similar to leukemoid reaction
* Can appear similar to [[leukemoid reaction]]
|-
|-
|[[Primary myelofibrosis (PMF)]]
|[[Primary myelofibrosis]] (PMF)  
|
|
* Clonal disorder of megakaryocytes
* Clonal disorder of megakaryocytes
Line 114: Line 114:
* [[Ruxolitinib]]
* [[Ruxolitinib]]
|
|
* Variable risk for development of acute leukemia
* Variable risk for development of [[acute leukemia]]
|-
|-
||[[Secondary polycythemia]]  
||Secondary polycythemia   
|
|
* [[Congestive heart failure]] (CHF)
* [[Congestive heart failure]] (CHF)
Line 123: Line 123:
* Smoking
* Smoking
* High altitude living
* High altitude living
* 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
|
|
* Headache
* [[Headache]]
* Fatigue
* [[Fatigue]]
* Shortness of breath
* [[Shortness of breath]]
|
|
* Elevated [[hemoglobin]]
* Elevated [[hemoglobin]]
Line 134: Line 134:
|
|
* [[Crackles]]
* [[Crackles]]
* Cyanosis
* [[Cyanosis]]
* Tachypnea
* [[Tachypnea]]
* Tachycardia
* [[Tachycardia]]
|
|
* Treatment of underlying cause of hypoxia
* Treatment of underlying cause of hypoxia
Line 143: Line 143:
* Steroids for ILD
* Steroids for ILD
* Continuous positive airway pressure for OSA
* Continuous positive airway pressure for OSA
* Smoking cessation or nicotine replacement
* [[Smoking cessation]] or nicotine replacement
* Surgical removal of tumors
* Surgical removal of tumors
|
|
Line 149: Line 149:
|-
|-
|}
|}


:<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>
:<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>

Revision as of 14:06, 21 February 2018

Polycythemia vera Microchapters

Home

Patient Information

Overview

Historical perspective

Classification

Pathophysiology

Causes

Differentiating Polycythemia vera from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural history, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Polycythemia vera differential diagnosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Polycythemia vera differential diagnosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Polycythemia vera differential diagnosis

CDC on Polycythemia vera differential diagnosis

Polycythemia vera differential diagnosis in the news

Blogs on Polycythemia vera differential diagnosis

Directions to Hospitals Treating Polycythemia vera

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.

Characteristic/Parameter 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
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
  • 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
Primary myelofibrosis (PMF)
  • Clonal disorder of megakaryocytes
  • Excess production TGF-beta and collagen
  • Fatigue
  • Abdominal pain
  • Early satiety
Secondary polycythemia
  • 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
[1][2][3]


References

  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.
  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.
  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.

Template:Hematology


Template:WikiDoc Sources