Polycythemia vera differential diagnosis: Difference between revisions
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Polycythemia vera must be differentiated from [[chronic myelogenous leukemia]], [[essential thrombocythemia]], and [[primary myelofibrosis]].<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> | Polycythemia vera must be differentiated from [[chronic myelogenous leukemia]], [[essential thrombocythemia]], and [[primary myelofibrosis]].<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> | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Polycythemia vera must be differentiated from the:<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. | ||
{| class="wikitable" | |||
!Characteristic/Parameter | |||
!Etiology | |||
!Symptoms | |||
!Laboratory abnormalities | |||
!Physical examination | |||
!Treatment | |||
!Other associated abnormalities | |||
|- | |||
||[[Polycythemia vera]] | |||
| | |||
* JAK2 mutation (V617F or exon 12 mutation) | |||
* 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]]| | |||
- | |||
||[[Chronic myeloid leukemia]] | |||
| | |||
* Abdominal pain | |||
* Infections | |||
* Fever | |||
| | |||
* [[Microcytic]] (<80 femtoliter) or normocytic | |||
| | |||
* Abnormal [[hemoglobin]] [[electrophoresis]] with elevated HbSS fraction | |||
* Sickled [red blood cells]] on peripheral blood smear | |||
* Elevated ferritin (due to frequent transfusions and iron overload) | |||
* Elevated [[red blood cell]] mass | |||
| | |||
* Vaso-occlusion in peripheral vascular beds | |||
* [[Jaundice]] from hemolysis | |||
* [[Hepatomegaly]] | |||
* [[Splenomegaly]] | |||
| | |||
* Folate supplemention | |||
* Hydroxyurea (to increase fetal [[hemoglobin]] production | |||
* Oxygen supplemention | |||
* [[Blood transfusion|Transfusion]] support | |||
* [[Iron]] chelation | |||
* [[Gene therapy]] if available | |||
* Crizanlizumab (P-selectin inhibitor) | |||
* Patient education and genetic counseling | |||
| | |||
* [[Extramedullary hematopoiesis]] | |||
* Pain crises | |||
* Acute chest syndrome | |||
|- | |||
|[[Iron deficiency anemia]] | |||
| | |||
* Loss of [[iron]] from occult bleeding | |||
* [[Gastrointestinal]] [[blood loss]] | |||
* [[Menstrual]] [[blood loss]] | |||
* Dietary iron deficiency | |||
| | |||
* [[Microcytic]] (<80 femtoliter) | |||
| | |||
* Low serum [[iron]] | |||
* Elevated [[transferrin]] | |||
* Low [[transferrin saturation]] | |||
* Low [[ferritin]] | |||
| | |||
* [[Pallor]] | |||
* [[Weakness]] | |||
* Positive [[occult blood]] testing (if [[Gastrointestinal bleeding|GI bleeding]]) | |||
| | |||
* Intravenous or oral iron supplementation | |||
* Blood transfusions | |||
| | |||
* [[Blood loss|Chronic blood loss]] | |||
|- | |||
|[[Hemolytic anemia]] | |||
| | |||
* Medication-induced | |||
* [[Autoimmunity]] | |||
* [[Red blood cell]] membrane defect | |||
* [[Red blood cell]] enzyme defect | |||
* Shear stress from mechanical valves | |||
| | |||
* [[Normocytic anemia|Normocytic]] (80-100 femtoliter) | |||
| | |||
* Indirect [[hyperbilirubinemia]] | |||
* [[Reticulocytosis]] | |||
* Low [[haptoglobin]] | |||
* Elevated [[LDH]] | |||
| | |||
* [[Pallor]] | |||
* [[Jaundice]] | |||
| | |||
* Removal of offending agent causing hemolysis | |||
* [[Steroids]] | |||
* Alternative [[immunosuppression]] | |||
| | |||
* [[HELLP syndrome]] | |||
* [[TTP]] | |||
* [[Chronic lymphocytic leukemia|CLL]] | |||
|- | |||
|[[Sideroblastic anemia]] | |||
| | |||
* [[Alcoholism]] | |||
* [[Lead poisoning]] | |||
* [[Vitamin B6 deficiency]] | |||
* [[Isoniazid]] | |||
* [[Chloramphenicol]] | |||
| | |||
* [[Microcytic anemia|Microcytic]] (<80 femtoliter) | |||
Or | |||
* [[Normocytic anemia|Normocytic]] (80-100 femtoliter) | |||
| | |||
* Ringed sideroblasts in [[bone marrow]] | |||
* Low [[vitamin B6]] level | |||
* High [[lead]] level | |||
| | |||
* [[Pallor]] | |||
* [[Muscle weakness|Weakness]] | |||
| | |||
* Removal of offending [[medication]] | |||
* High-dose [[vitamin B6]] (up to 200mg daily) | |||
* Avoidance of [[splenectomy]] | |||
* Symptomatic [[Blood transfusion|transfusion]] support with [[iron]] [[chelation]] as needed | |||
| | |||
* [[Myelodysplastic syndrome]] | |||
* [[Myeloproliferative neoplasm]] | |||
* [[Iron overload]] | |||
|- | |||
|[[Anemia of chronic disease]] | |||
| | |||
* [[Chronic kidney disease]] | |||
* [[Rheumatologic disease]] | |||
* [[Cancer]] | |||
* [[HIV]] | |||
* Chronic infection; excess release of [[IL-1]] and [[IL-6]] | |||
| | |||
* [[Normocytic anemia|Normocytic]] (80-100 femtoliter) | |||
| | |||
* Elevated [[ESR]] and [[CRP]] | |||
* Elevated [[hepcidin]] | |||
* Low serum [[iron]] | |||
* Low [[transferrin]] | |||
* Elevated [[ferritin]] | |||
| | |||
* [[Pallor]] | |||
* [[Weakness]] | |||
| | |||
* Treatment of the underlying cause; [[erythropoiesis]]-stimulating agents | |||
* Supportive [[Red blood cell transfusion|red blood cell transfusions]] | |||
| | |||
* [[Inflammatory bowel disease]] | |||
|- | |||
|[[Erythropoietin]] deficiency | |||
| | |||
* [[Chronic kidney disease]] or other [[renal dysfunction]] | |||
| | |||
* [[Normocytic anemia|Normocytic]] (80-100 femtoliter) | |||
| | |||
* Low [[Erythropoietin|erythropoietin level]] | |||
| | |||
* [[Pallor]] | |||
* [[Weakness]] | |||
* Signs of [[chronic kidney disease]] | |||
| | |||
* [[Epoetin alfa]] 50-100 units/kg 3 times weekly | |||
* Darbepoietin 0.45 mcg/kg weekly or 0.75 mcg/kg every 2 weeks<ref name="pmid28626220">{{cite journal| author=Platzbecker U, Symeonidis A, Oliva EN, Goede JS, Delforge M, Mayer J et al.| title=A phase 3 randomized placebo-controlled trial of darbepoetin alfa in patients with anemia and lower-risk myelodysplastic syndromes. | journal=Leukemia | year= 2017 | volume= 31 | issue= 9 | pages= 1944-1950 | pmid=28626220 | doi=10.1038/leu.2017.192 | pmc=5596208 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28626220 }} </ref> | |||
| | |||
* [[Dialysis]] dependence | |||
* [[Myelodysplastic syndrome]] | |||
|- | |||
|[[Vitamin B12 deficiency|Vitamin B12]] or [[folate deficiency]] | |||
| | |||
* [[Pernicious anemia]] | |||
* [[Diphyllobothrium latum infection]] | |||
* [[Nutritional deficiency]] | |||
* [[Crohn's disease|Crohn's disease of terminal ileum]] | |||
| | |||
* [[Macrocytic anemia|Macrocytic]] (>100 femtoliter) | |||
| | |||
* Low vitamin B12 or folate level | |||
* [[Megaloblastic anemia]] with hypersegmented [[neutrophils]] | |||
| | |||
* [[Numbness]] | |||
* [[Weakness]] | |||
* [[Tingling]] | |||
* [[Paresthesias]] | |||
| | |||
* [[Vitamin B12]] 1000mcg daily | |||
* [[Folate]] 1mg daily | |||
| | |||
* [[Neuropathy]] | |||
|} | |||
:<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> | |||
*[[Chronic myelogenous leukemia]] | *[[Chronic myelogenous leukemia]] | ||
*[[Essential thrombocythemia]] | *[[Essential thrombocythemia]] |
Revision as of 23:00, 28 January 2018
Polycythemia vera Microchapters |
Diagnosis |
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Treatment |
Case Studies |
Polycythemia vera differential diagnosis On the Web |
American Roentgen Ray Society Images of Polycythemia vera differential diagnosis |
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]
Overview
Polycythemia vera must be differentiated from chronic myelogenous leukemia, essential thrombocythemia, and primary myelofibrosis.[1][2]
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 |
|
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- |
Chronic myeloid leukemia |
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Iron deficiency anemia |
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Hemolytic anemia |
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Sideroblastic anemia |
Or
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Anemia of chronic disease |
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Erythropoietin deficiency |
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Vitamin B12 or folate deficiency |
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References
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 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.
- ↑ Platzbecker U, Symeonidis A, Oliva EN, Goede JS, Delforge M, Mayer J; et al. (2017). "A phase 3 randomized placebo-controlled trial of darbepoetin alfa in patients with anemia and lower-risk myelodysplastic syndromes". Leukemia. 31 (9): 1944–1950. doi:10.1038/leu.2017.192. PMC 5596208. PMID 28626220.
- ↑ 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.