Difference between revisions of "Polycythemia vera"

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*Can be asymptomatic or symptomatic
 
*Can be asymptomatic or symptomatic
  
==Clinical Presentation==
+
==Clinical Features==
 
*Severe burning pain in the hands or feet accompanied by a reddish or bluish coloration of the skin
 
*Severe burning pain in the hands or feet accompanied by a reddish or bluish coloration of the skin
 
*Suspected with any combination of the following: <ref>http://emedicine.medscape.com/article/205114-differential</ref>
 
*Suspected with any combination of the following: <ref>http://emedicine.medscape.com/article/205114-differential</ref>
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**Pruritus after bathing
 
**Pruritus after bathing
  
==Diagnostic Criteria<ref>Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,</ref>==
+
==Differential Diagnosis==
 +
*Essential thrombocytosis (ET)
 +
*Chronic myelogenous leukemia (CML)
 +
*Leukemoid reactions
 +
*Hypoxia
 +
 
 +
==Diagnosis==
 +
===Workup===
 +
*CBC
 +
*Chem 7
 +
*ESR
 +
*EPO
 +
*Bone Marrow Bx sometimes needed
 +
 
 +
 
 +
===Evaluation<ref>Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,</ref>===
 
*Criteria 1: All category A
 
*Criteria 1: All category A
 
*Criteria 2: First 3 of category A and any 2 of category B
 
*Criteria 2: First 3 of category A and any 2 of category B
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|  || Vit B12 >900
 
|  || Vit B12 >900
 
|}
 
|}
==DDx==
 
*Essential thrombocytosis (ET)
 
*Chronic myelogenous leukemia (CML)
 
*Leukemoid reactions
 
*Hypoxia
 
 
==Labs==
 
*CBC
 
*Chem 7
 
*ESR
 
*EPO
 
*Bone Marrow Bx sometimes needed
 
  
 
==Treatment==
 
==Treatment==
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==Also See==
 
==Also See==
  
 
+
==References==
==Sources==
 
 
<references/>
 
<references/>
  
 
[[Category:Heme/Onc]]
 
[[Category:Heme/Onc]]

Revision as of 07:04, 29 August 2015

Background

  • Chronic myeloproliferative disorder
  • Abnormal proliferation is seen in all 3 cell lines
  • Typically in elderly
  • Can be asymptomatic or symptomatic

Clinical Features

  • Severe burning pain in the hands or feet accompanied by a reddish or bluish coloration of the skin
  • Suspected with any combination of the following: [1]
    • Abnormally elevated hemoglobin levels (>18 g/dL in men; 16 g/dL in women)
    • Normal oxygen saturation
    • Bleeding complications
    • Portal vein thrombosis
    • Splenomegaly
    • Plethora
    • Pruritus after bathing

Differential Diagnosis

  • Essential thrombocytosis (ET)
  • Chronic myelogenous leukemia (CML)
  • Leukemoid reactions
  • Hypoxia

Diagnosis

Workup

  • CBC
  • Chem 7
  • ESR
  • EPO
  • Bone Marrow Bx sometimes needed


Evaluation[2]

  • Criteria 1: All category A
  • Criteria 2: First 3 of category A and any 2 of category B
Category A Category B
Increased RBC Mass (Men >18.5, Women >16.5) Thrombocytosis (>400,000)
Normal SPO2 (>92%) Leukocytosis (>12,000)
Splenomegaly Leukocyte Alk Phos >100
Vit B12 >900

Treatment

  • Phlebotomy
  • ASA 81 mg
  • Myelosuppressive agents (hydroxyurea, interferon alpha)

Also See

References

  1. http://emedicine.medscape.com/article/205114-differential
  2. Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,