Polycythemia vera differential diagnosis: Difference between revisions

	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

VisualWikitext

Revision as of 14:25, 27 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	Signs	Laboratory abnormalities	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	Ruddy face Splenomegaly	↑ Hemoglobin ↑ RBC mass ↑ WBC ↑ Platelet Panmyelosis	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	Splenomegaly Bruising	↑ Platelet	Aspirin Hydroxyurea Anagrelide	Paradoxical bleeding 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	Splenomegaly	↑ RBC mass ↑ WBC ↑ Platelet ↑ Proportion of metamyelocytes and other white blood cells at various stages of maturation	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	Splenomegaly Hepatomegaly Pallor Bruising	↓ Hemoglobin ↓ WBC ↓ Platelet	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	Crackles Cyanosis Tachypnea Tachycardia	↑ Hemoglobin ↑ Erythropoietin level	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.

[1]

[2]

[3]

@@ Line 20: / Line 20: @@
 | align="left" style="background:#F5F5F5;" |
 * [[JAK2]] mutation (V617F or exon 12 mutation) in more than 95% of cases
-* Autonomous erythrocyte production
+* Autonomous [[Red blood cell|erythrocyte]] production
 | align="left" style="background:#F5F5F5;" |
 * [[Erythromelagia]]
@@ Line 29: / Line 29: @@
 * [[Splenomegaly]]
 | align="left" style="background:#F5F5F5;" |
-* Elevated [[hemoglobin]]
+* ↑ [[Hemoglobin]]
-* Elevated [[red blood cell]] mass
+* ↑ [[Red blood cell|RBC]] mass
-* Elevated [[white blood cell]] count and [[platelet]] count in some cases (panmyelosis)
+* ↑ [[White blood cells|WBC]]
+* ↑ [[Platelet]]
+* Panmyelosis
 | align="left" style="background:#F5F5F5;" |
 * [[Aspirin]]
@@ Line 46: / Line 48: @@
 ! align="center" style="background:#DCDCDC;" |[[Essential thrombocythemia]] (ET)
 | align="left" style="background:#F5F5F5;" |
-* Clonal proliferation of megakaryocytes
+* Clonal proliferation of [[Megakaryocyte|megakaryocytes]]
 * Excess [[platelet]] production
-* Caused by JAK2 mutation in 50% of cases
+* Caused by [[Janus kinase|JAK2]] mutation in 50% of cases
 * Caused by CALR mutation in some cases
 | align="left" style="background:#F5F5F5;" |
-* Stroke-like symptoms
+* [[Stroke]]-like symptoms
 * [[Fatigue]]
-* Bleeding due to dysfunctional platelets and acquired [[von Willebrand disease]]
+* [[Bleeding]] due to dysfunctional platelets and acquired [[von Willebrand disease]]
 | align="left" style="background:#F5F5F5;" |
 * [[Splenomegaly]]
-* Bruising
+* [[Bruise|Bruising]]
 | align="left" style="background:#F5F5F5;" |
-* Elevated total [[platelet]] count
+* ↑ [[Platelet]]
 | align="left" style="background:#F5F5F5;" |
 * [[Aspirin]]
-* [[Hydroyurea]]
+* [[Hydroxyurea]]
 * [[Anagrelide]]
 | align="left" style="background:#F5F5F5;" |
-* Paradoxical bleeding can occur when platelet count is above 1,000,000 cells/microliter due to acquired von Willebrand disease
+* Paradoxical [[bleeding]]
-* Can progress to post-ET myelofibrosis
+* Can progress to post-ET [[myelofibrosis]]
-* Can develop into acute leukemia
+* Can develop into [[acute leukemia]]
 |-
 ! align="center" style="background:#DCDCDC;" |[[Chronic myeloid leukemia]] (CML)
 | align="left" style="background:#F5F5F5;" |
 * Reciprocal translocation of chromosomes 9 and 22
-* Production of BCR-Abl kinase, which drives cell proliferation
+* Production of [[BCR/ABL|BCR-Abl kinase]], which drives cell proliferation
 | align="left" style="background:#F5F5F5;" |
 * [[Abdominal pain]]
@@ Line 79: / Line 81: @@
 * [[Splenomegaly]]
 | align="left" style="background:#F5F5F5;" |
-* Elevated total [[white blood cell count]]
+* ↑ [[Red blood cell|RBC]] mass
-* Elevated [[red blood cell]] count and [[platelet]] count occasionally
+* ↑ [[White blood cells|WBC]]
-* Increased proportion of metamyelocytes and other [[white blood cells]] at various stages of maturation
+* ↑ [[Platelet]]
+* ↑ Proportion of metamyelocytes and other [[white blood cells]] at various stages of maturation
 | align="left" style="background:#F5F5F5;" |
 * [[Imatinib]]
@@ Line 106: / Line 109: @@
 * [[Bruising]]
 | align="left" style="background:#F5F5F5;" |
-* Decreased [[hemoglobin]] (anemia)
+* ↓ [[Hemoglobin]]
-* Decreased [[platelet]] count (thrombocytopenia)
+* ↓ [[White blood cells|WBC]]
-* Decreased [[white blood cell]] count (leukopenia)
+* ↓ [[Platelet]]
 | align="left" style="background:#F5F5F5;" |
 * [[Hydroxyurea]]
@@ Line 121: / Line 124: @@
 * [[Interstitial lung disease]] (ILD)
 * [[Obstructive sleep apnea]] (OSA)
-* Smoking
+* [[Smoking]]
 * High altitude living
 * Ectopic [[erythropoietin]] production from tumor (renal cell carcinoma or hepatocellular carcinoma)
@@ Line 135: / Line 138: @@
 * [[Tachycardia]]
 | align="left" style="background:#F5F5F5;" |
-* Elevated [[hemoglobin]]
+* ↑ [[Hemoglobin]]
-* Increased [[erythropoietin]] level
+* ↑ [[Erythropoietin]] level
 | align="left" style="background:#F5F5F5;" |
 * Treatment of underlying cause of [[hypoxia]]

Polycythemia vera differential diagnosis: Difference between revisions

Revision as of 14:25, 27 August 2018

Overview

Differential Diagnosis

References

Navigation menu