Polycythemia vera differential diagnosis: Difference between revisions

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* Variable risk for development of acute leukemia
* Variable risk for development of acute leukemia
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|-
|[[Hemolytic anemia]] 
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* Medication-induced
* [[Autoimmunity]]
* [[Red blood cell]] membrane defect
* [[Red blood cell]] enzyme defect
* Shear stress from mechanical valves
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* [[Normocytic anemia|Normocytic]] (80-100 femtoliter)
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* Indirect [[hyperbilirubinemia]]
* [[Reticulocytosis]]
* Low [[haptoglobin]]
* Elevated [[LDH]]
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* [[Pallor]]
* [[Jaundice]]
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* Removal of offending agent causing hemolysis
* [[Steroids]]
* Alternative [[immunosuppression]]
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* [[HELLP syndrome]]
* [[TTP]]
* [[Chronic lymphocytic leukemia|CLL]]
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|[[Sideroblastic anemia]]
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* [[Alcoholism]]
* [[Lead poisoning]]
* [[Vitamin B6 deficiency]]
* [[Isoniazid]]
* [[Chloramphenicol]]
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* [[Microcytic anemia|Microcytic]] (<80 femtoliter)
Or
* [[Normocytic anemia|Normocytic]] (80-100 femtoliter)
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* Ringed sideroblasts in [[bone marrow]]
* Low [[vitamin B6]] level
* High [[lead]] level
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* [[Pallor]]
* [[Muscle weakness|Weakness]]
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* 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
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* [[Myelodysplastic syndrome]]
* [[Myeloproliferative neoplasm]]
* [[Iron overload]]
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|[[Anemia of chronic disease]]
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* [[Chronic kidney disease]]
* [[Rheumatologic disease]]
* [[Cancer]]
* [[HIV]]
* Chronic infection; excess release of [[IL-1]] and [[IL-6]]
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* [[Normocytic anemia|Normocytic]] (80-100 femtoliter)
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* Elevated [[ESR]] and [[CRP]]
* Elevated [[hepcidin]]
* Low serum [[iron]]
* Low [[transferrin]]
* Elevated [[ferritin]]
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* [[Pallor]]
* [[Weakness]]
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* Treatment of the underlying cause; [[erythropoiesis]]-stimulating agents
* Supportive [[Red blood cell transfusion|red blood cell transfusions]]
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* [[Inflammatory bowel disease]]
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|[[Erythropoietin]] deficiency
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* [[Chronic kidney disease]] or other [[renal dysfunction]]
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* [[Normocytic anemia|Normocytic]] (80-100 femtoliter)
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* Low [[Erythropoietin|erythropoietin level]]
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* [[Pallor]]
* [[Weakness]]
* Signs of [[chronic kidney disease]]
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* [[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>
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* [[Dialysis]] dependence
* [[Myelodysplastic syndrome]]
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|[[Vitamin B12 deficiency|Vitamin B12]] or [[folate deficiency]]
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* [[Pernicious anemia]]
* [[Diphyllobothrium latum infection]]
* [[Nutritional deficiency]]
* [[Crohn's disease|Crohn's disease of terminal ileum]]
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* [[Macrocytic anemia|Macrocytic]] (>100 femtoliter)
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* Low vitamin B12 or folate level
* [[Megaloblastic anemia]] with  hypersegmented [[neutrophils]]
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* [[Numbness]]
* [[Weakness]]
* [[Tingling]]
* [[Paresthesias]]
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* [[Vitamin B12]] 1000mcg daily
* [[Folate]] 1mg daily
|
* [[Neuropathy]]
|}
|}


Revision as of 23:27, 28 January 2018

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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 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 (PV)
  • JAK2 mutation (V617F or exon 12 mutation)
  • Autonomous erythrocyte production
  • Erythromelagia
  • Headache
  • Stroke-like symptoms
  • Aspirin
  • Ruxolitinib
  • Hydroxyurea
  • Phlebotomy
  • Interferon-alpha
Essential thrombocythemia (ET)
  • Clonal proliferation of megakaryocytes
  • Excess platelet production
  • Occasionally caused by JAK2 mutation
  • Stroke-like symptoms
  • Fatigue
  • Bleeding due to dysfunctional platelets and acquired von Willebrand disease
  • 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
  • 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
  • Variable risk for development of acute leukemia


[1][2][3]

References

  1. ↑ 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. ↑ 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.
  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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