Difference between revisions of "Polycythemia vera"

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==Background==
 
==Background==
*Chronic myeloproliferative disorder
+
*Chronic [[myeloproliferative disorders|myeloproliferative disorder]]
 
*Abnormal proliferation is seen in all 3 cell lines
 
*Abnormal proliferation is seen in all 3 cell lines
 
*Typically in elderly
 
*Typically in elderly
 
*Can be asymptomatic or symptomatic
 
*Can be asymptomatic or symptomatic
 +
*Commonly caused by JAK2 mutation
  
==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>
**Abnormally elevated hemoglobin levels (>18 g/dL in men; 16 g/dL in women)
+
**Abnormally ''elevated'' hemoglobin levels (>18 g/dL in men; 16 g/dL in women)
 
**Normal oxygen saturation
 
**Normal oxygen saturation
 
**Bleeding complications
 
**Bleeding complications
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**Splenomegaly
 
**Splenomegaly
 
**Plethora
 
**Plethora
**Pruritus after bathing
+
**[[Pruritus]] after bathing
 +
*May develop [[hyperviscosity syndrome]]
  
==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==
 +
*Primary polycythemia ([[myeloproliferative disorders]])
 +
**[[Polycythemia vera]]
 +
**[[Essential thrombocytosis]] (ET)
 +
**[[Chronic myelogenous leukemia]] (CML)
 +
**[[Leukemoid Reaction]]
 +
**[[Myelofibrosis]]
 +
*Secondary polycythemia (due to increased EPO)
 +
**Chronic [[hypoxemia]] (e.g. [[COPD]], physiologic [[altitude]] adaptations or [[chronic mountain sickness]])
 +
**Carboxyhemoglobin (chronic smokers)
 +
**Renal cyst or hydronephrosis
 +
**EPO-secreting tumors (RCC, [[hepatocellular carcinoma]])
 +
**Anabolic steroid abuse
 +
**Abnormal hemoglobins
 +
 
 +
==Evaluation==
 +
===Workup===
 +
*CBC
 +
*Chem 7
 +
*ESR
 +
*EPO decreased
 +
*Bone marrow biopsy 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==
 
*Phlebotomy
 
*ASA 81 mg
 
*Myelosuppressive agents
 
  
==Also See==
+
==Management==
 +
*Phlebotomy with goal hematocrit < 45%<ref>Marchioli R et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med.  2013; 368(1):22-33.</ref>
 +
**Patients with severe plethora, with [[AMS]] or vascular compromise can be bled vigorously with removal of 500 mL of whole blood rapidly
 +
**Otherwise, gradual phlebotomy of 500-1000 mL over 24 hours is preferred in less emergent cases
 +
*[[ASA]] 81mg
 +
*Myelosuppressive agents ([[hydroxyurea]], [[interferon-α]])
 +
*If presenting with [[hyperviscosity syndrome]]:
 +
**[[IVF]] and plebotomy
  
 +
==See Also==
 +
*[[Hyperviscosity syndrome]]
  
==Sources==
+
==References==
http://emedicine.medscape.com/article/205114-differential
 
 
<references/>
 
<references/>
  
 
[[Category:Heme/Onc]]
 
[[Category:Heme/Onc]]

Latest revision as of 15:08, 12 February 2021

Background

  • Chronic myeloproliferative disorder
  • Abnormal proliferation is seen in all 3 cell lines
  • Typically in elderly
  • Can be asymptomatic or symptomatic
  • Commonly caused by JAK2 mutation

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
  • May develop hyperviscosity syndrome

Differential Diagnosis

Evaluation

Workup

  • CBC
  • Chem 7
  • ESR
  • EPO decreased
  • Bone marrow biopsy 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

Management

  • Phlebotomy with goal hematocrit < 45%[3]
    • Patients with severe plethora, with AMS or vascular compromise can be bled vigorously with removal of 500 mL of whole blood rapidly
    • Otherwise, gradual phlebotomy of 500-1000 mL over 24 hours is preferred in less emergent cases
  • ASA 81mg
  • Myelosuppressive agents (hydroxyurea, interferon-α)
  • If presenting with hyperviscosity syndrome:
    • IVF and plebotomy

See Also

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,
  3. Marchioli R et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013; 368(1):22-33.