Polycythemia vera differential diagnosis: Difference between revisions

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Revision as of 17:36, 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	Mutation		Etiology	Symptoms					Signs					laboratory data						Treatment	Associated findings
	Mutation			Symptoms					Signs					CBC				Erythropoietin level	PBS
	JAK2 mutation	CALR mutation		Fatigue	Headache	Bleeding	Pain	Other symptoms	Appearance	Fever	Tenderness	Splenomegaly	Other signs	Hb	RBC	WBC	Plt	Erythropoietin level	PBS
Polycythemia vera (PV)	> 95%		Autonomous erythrocyte production		+	-	Erythromelagia	Stroke-like symptoms	Ruddy face	-	-	+	NA	↑	↑ RBC mass	Nl to ↑	Nl to ↑			Aspirin Ruxolitinib Hydroxyurea Phlebotomy Interferon-alpha	Stroke Venous thrombosis Myelofibrosis Acute leukemia
Essential thrombocythemia (ET)	50%	+/-	Clonal proliferation of megakaryocytes Excess platelet production	+		+ (paradoxical)		Stroke-like symptoms				+	Bruises				↑ (dysfunctional platelets)			Aspirin Hydroyurea Anagrelide	Acquired von Willebrand disease Myelofibrosis Acute leukemia
Chronic myeloid leukemia (CML)	-	-	Reciprocal translocation of chromosomes 9 and 22 Production of BCR-Abl kinase	+	+	+	Abdominal pain			+		+		↑	↑	↑	↑		Elevated 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	+		+	Abdominal pain	Early satiety	Pallor			+	Hepatomegaly 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) Chronic hypoxia Ectopic erythropoietin production from tumor (renal cell carcinoma or hepatocellular carcinoma)	+	+	-	Depends on etiology	Shortness of breath	Cyanosis				Crackles Tachypnea Tachycardia	↑				↑		Treatment of underlying cause	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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@@ Line 14: / Line 14: @@
 ! colspan="5" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptoms
 ! colspan="5" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Signs
-! colspan="7" style="background: #4479BA; color: #FFFFFF; text-align: center;" |laboratory data
+! colspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" |laboratory data
 ! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Treatment
 ! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
@@ Line 21: / Line 21: @@
 ! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Erythropoietin level
 ! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |PBS
-! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |PT/PTT
 |-
 ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |JAK2 mutation
@@ Line 45: / Line 44: @@
 | align="center" style="background:#F5F5F5;" |Autonomous erythrocyte 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="left" style="background:#F5F5F5;" |
 * [[Erythromelagia]]
@@ Line 60: / Line 59: @@
 | 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;" |
@@ Line 77: / Line 75: @@
 ! align="center" style="background:#DCDCDC;" |[[Essential thrombocythemia]] (ET)
 | align="center" style="background:#F5F5F5;" |50%
-| align="center" style="background:#F5F5F5;" |+/-
+| align="center" style="background:#F5F5F5;" | +/-
 | align="left" style="background:#F5F5F5;" |
 * Clonal proliferation of megakaryocytes
@@ Line 83: / Line 81: @@
 | align="center" style="background:#F5F5F5;" | +
 | align="center" style="background:#F5F5F5;" |
-| align="center" style="background:#F5F5F5;" |+ (paradoxical)
+| align="center" style="background:#F5F5F5;" | + (paradoxical)
 | align="center" style="background:#F5F5F5;" |
 | align="left" style="background:#F5F5F5;" |
@@ Line 90: / Line 88: @@
 | 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;" |[[Bruises]]
 | align="center" style="background:#F5F5F5;" |
@@ Line 96: / Line 94: @@
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |↑ (dysfunctional platelets)
-| align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
 | align="center" style="background:#F5F5F5;" |
@@ Line 109: / Line 106: @@
 |-
 ! align="center" style="background:#DCDCDC;" |[[Chronic myeloid leukemia]] (CML)
-| 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;" |
 * Reciprocal translocation of chromosomes 9 and 22
 * Production of BCR-Abl kinase
-| 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;" |[[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="center" style="background:#F5F5F5;" |↑
@@ Line 130: / Line 127: @@
 | 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;" |
 | align="left" style="background:#F5F5F5;" |
 * [[Imatinib]]
@@ Line 164: / Line 160: @@
 | 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;" |
@@ Line 175: / Line 170: @@
 |-
 ! align="center" style="background:#DCDCDC;" |Secondary polycythemia
-| 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;" |
 * [[Congestive heart failure]] (CHF)
@@ Line 183: / Line 178: @@
 * Chronic [[hypoxia]]
 * Ectopic [[erythropoietin]] production from tumor (renal cell carcinoma or hepatocellular carcinoma)
-| 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
@@ Line 202: / Line 197: @@
 | 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;" |
-* Treatment of underlying cause of [[hypoxia]]
+* Treatment of underlying cause
-* Supplemental oxygen
-* Diuresis for [[CHF]]
-* Steroids for ILD
-* Continuous positive airway pressure for OSA
-* [[Smoking cessation]] or nicotine replacement
-* Surgical removal of tumors
 | align="left" style="background:#F5F5F5;" |
 * Variable manifestations given the diverse etiologies

Polycythemia vera differential diagnosis: Difference between revisions

Revision as of 17:36, 20 August 2018

Overview

Differential Diagnosis

References

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