Thrombocytosis: Difference between revisions
 
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==Background==
==Background==
*Defined as a platelet count >450,000/microL.
*Defined as a platelet count >450,000/microL.
*Can be reactive or autonomous.
**Reactive thrombocytosis (RT) is the most common cause of thrombocytosis, accounting for 85% of cases. RT is a reaction to another process, such as inflammation, infection, cancer, or iron deficiency.  RT rarely causes complications even with extremely elevated platelet counts (>1,000,000/microL).
**Autonomous thrombocytosis (AT) accounts for 15% of cases.  AT is a primary problem that results from [[myeloproliferative disorders]] or [[myelodysplastic disorders]], such as essential [[thrombocytopenia]] or [[polycythemia vera]].  Complications, such as bleeding and/or thrombosis, are more likely with AT than with RT so it is clinically important to differentiate reactive thrombocytosis versus autonomous thrombocytosis.  There are no diagnostic studies to differentiate RT versus AT.  <ref>Schafer, "Thrombocytosis," N Engl J Med 2004;350:1211-9.</ref>


==Clinical Features==
===Reactive thrombocytosis (RT)===
*Generally asymptomatic and found on routine lab testing.
*Most common cause of thrombocytosis, accounting for 85% of cases.
*Complications are seen in AT disorders and include thrombosis, bleeding, or vasomotor symptoms.  Thrombotic complications are the leading cause of morbidity and mortality. Thrombosis commonly occurs in arteries (stroke, TIA, MI, unstable angina), but can also occur in the venous system ([[DVT]], [[PE]], [[Budd-Chiari syndrome]]).  Serious bleeding is a less common complication and is generally seen in the nasal and bucchal mucosa and the GI tract.
*A reaction to another process, such as inflammation, infection, cancer, or iron deficiency. 
*Vasomotor symptoms are due to microvascular disturbances and include: headache, lightheadedness, syncope, acral paresthesia, atypical chest pain, livedo reticularis, erythromelalgia, and transient visual disturbances. <ref>Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.</ref>
*Rarely causes complications even with extremely elevated platelet counts (>1,000,000/microL).
 
===Autonomous thrombocytosis (AT)<ref>Schafer, "Thrombocytosis," N Engl J Med 2004;350:1211-9.</ref>===
*Accounts for 15% of cases
*A primary problem that results from [[myeloproliferative disorders]] or [[myelodysplastic syndrome]]s, such as essential [[thrombocytopenia]] or [[polycythemia vera]].
*Complications, such as bleeding and/or thrombosis, are more likely with AT than with RT so it is clinically important to differentiate reactive thrombocytosis versus autonomous thrombocytosis. 
*There are no diagnostic studies to differentiate RT versus AT.
 
==Clinical Features<ref>Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.</ref>==
''Generally asymptomatic and found on routine lab testing; complications are typically from AT disorders''
*Thrombosis (leading cause of morbidity and mortality)
**Arteries (more common)
***[[Stroke]], [[TIA]], [[MI]], [[unstable angina]]
**Venous system
***[[DVT]], [[PE]], [[Budd-Chiari syndrome]]
*Serious bleeding (less common)
**Generally in the nasal and bucchal mucosa and the GI tract
*Vasomotor symptoms (due to microvascular disturbances)
**[[Headache]], lightheadedness, [[syncope]], acral [[paresthesia]], atypical [[chest pain]], livedo reticularis, erythromelalgia, and transient [[visual disturbances]]


==Differential Diagnosis<ref>Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.</ref>==
==Differential Diagnosis<ref>Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.</ref>==
===Reactive thrombocytosis===
===Reactive thrombocytosis===
* Acute blood loss
* Acute [[hemorrhage|blood loss]]
* Acute hemolytic anemia
* Acute [[hemolytic anemia]]
* Iron deficiency anemia
* Iron deficiency [[anemia]]
* Treatment of [[vitamin B12 deficiency]]
* Treatment of [[vitamin B12 deficiency]]
*Rebound effect after thrombocytopenia treatment
* Rebound effect after [[thrombocytopenia]] treatment
* Metastatic cancer
* Metastatic cancer
* [[Lymphoma]]
* [[Lymphoma]]
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* POEMS syndrome
* POEMS syndrome
* Thermal [[burn]]
* Thermal [[burn]]
* [[MI ]]
* [[MI]]
* Severe trauma
* Severe [[trauma]]
* [[Acute pancreatitis]]
* [[Acute pancreatitis]]
* Post-surgery, especially splenectomy
* Post-surgery, especially [[splenectomy]]
* CABG
* CABG
* [[TB]]
* [[TB]]
* Acute bacterial/viral infections
* Acute [[bacterial disease|bacterial]]/[[viruses|viral]] infections
* Asplenia
* [[asplenic patient|Asplenia]]
* [[Allergic reaction]]
* [[Allergic reaction]]
* Medication reactions: vincristine, epinephrine, glucocorticoids, IL-1B, ATRA, thrombopoietin, LMWH (uptodate)
* Medication reactions: vincristine, [[epinephrine]], [[glucocorticoids]], IL-1B, ATRA, thrombopoietin, [[LMWH]]<ref>uptodate</ref>


===Autonomous thrombocytosis===
===Autonomous thrombocytosis===
* Essential thrombocytopenia
* [[Essential thrombocytosis]]
* [[Polycythemia vera]]
* [[Polycythemia vera]]
* Mixed myelodysplastic and/or myeloproliferative syndrome
* Mixed [[myelodysplastic syndrome|myelodysplastic]] and/or [[myeloproliferative disorders|myeloproliferative syndrome]]


===Spurious (false) thrombocytosis===
===Spurious (false) thrombocytosis===
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====Asymptomatic====
====Asymptomatic====
*81mg [[ASA]] PO daily.
*81mg [[ASA]] PO daily.
*If high risk  for thrombotic event (>60 yrs, history of thrombosis, or known JAK2 mutation), consider 15mg/kg hydroxyurea PO daily.  Consult if available.   
*If high risk  for thrombotic event (>60 yrs, history of thrombosis, or known JAK2 mutation), consider 15mg/kg [[hydroxyurea]] PO daily.  Consult if available.   
*Second line agents include IFN-alpha, anagrelide, and pipobroman.
*Second line agents include [[interferon-α]], anagrelide, and pipobroman.


====Thrombosis due to AT====
====Thrombosis due to AT====
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*81mg [[ASA]] PO daily.
*81mg [[ASA]] PO daily.
*Consult heme/onc
*Consult heme/onc
*Consider 3-5 million U IFN alpha SQ daily if <40 years old.  15mg/kg hydroxyurea PO daily if >40 years old.  Goal platelet count <400,000/microL.
*Consider 3-5 million U [[interferon-α]] SQ daily if <40 years old.  15mg/kg [[hydroxyurea]] PO daily if >40 years old.  Goal platelet count <400,000/microL.
*Platelet apheresis if platelet count >800,000/microL.
*Platelet apheresis if platelet count >800,000/microL.


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*Discontinue antiplatelet medications.
*Discontinue antiplatelet medications.
*Consult.
*Consult.
*Consider 3-5 million U IFN alpha SQ daily if <40 years old.  15mg/kg hydroxyurea PO daily if >40 years old.  Goal platelet count <400,000/microL.<ref>Bleeker, "Thrombocytosis: Diagnostic Evaluation, Thrombotic Risk Stratification, and Risk-Based Management Strategies," Thrombosis. 2011; 2011: 536062. Published online 2011 Jun 8. doi:10.1155/2011/536062.</ref>
*Consider 3-5 million U [[interferon-α]] SQ daily if <40 years old.  15mg/kg [[hydroxyurea]] PO daily if >40 years old.  Goal platelet count <400,000/microL.<ref>Bleeker, "Thrombocytosis: Diagnostic Evaluation, Thrombotic Risk Stratification, and Risk-Based Management Strategies," Thrombosis. 2011; 2011: 536062. Published online 2011 Jun 8. doi:10.1155/2011/536062.</ref>


==Disposition==
==Disposition==

Latest revision as of 23:46, 30 September 2019

Background

  • Defined as a platelet count >450,000/microL.

Reactive thrombocytosis (RT)

  • Most common cause of thrombocytosis, accounting for 85% of cases.
  • A reaction to another process, such as inflammation, infection, cancer, or iron deficiency.
  • Rarely causes complications even with extremely elevated platelet counts (>1,000,000/microL).

Autonomous thrombocytosis (AT)[1]

  • Accounts for 15% of cases
  • A primary problem that results from myeloproliferative disorders or myelodysplastic syndromes, such as essential thrombocytopenia or polycythemia vera.
  • Complications, such as bleeding and/or thrombosis, are more likely with AT than with RT so it is clinically important to differentiate reactive thrombocytosis versus autonomous thrombocytosis.
  • There are no diagnostic studies to differentiate RT versus AT.

Clinical Features[2]

Generally asymptomatic and found on routine lab testing; complications are typically from AT disorders

Differential Diagnosis[3]

Reactive thrombocytosis

Autonomous thrombocytosis

Spurious (false) thrombocytosis

  • Mixed cryoglobulinemia
  • Cytoplasmic fragments
  • Bacteremia

Evaluation

Workup

  • Labs
    • CBC, ESR, CRP, iron studies, LDH
  • CXR
  • Fecal occult blood test

Diagnosis

  • platelet count >450,000/microL.

Management

Reactive thrombocytosis

  • Treat underlying disease.

Autonomous thrombocytosis

Asymptomatic

  • 81mg ASA PO daily.
  • If high risk for thrombotic event (>60 yrs, history of thrombosis, or known JAK2 mutation), consider 15mg/kg hydroxyurea PO daily. Consult if available.
  • Second line agents include interferon-α, anagrelide, and pipobroman.

Thrombosis due to AT

  • Anticoagulation with LMWH.
  • 81mg ASA PO daily.
  • Consult heme/onc
  • Consider 3-5 million U interferon-α SQ daily if <40 years old. 15mg/kg hydroxyurea PO daily if >40 years old. Goal platelet count <400,000/microL.
  • Platelet apheresis if platelet count >800,000/microL.

Bleeding due to AT

  • Discontinue antiplatelet medications.
  • Consult.
  • Consider 3-5 million U interferon-α SQ daily if <40 years old. 15mg/kg hydroxyurea PO daily if >40 years old. Goal platelet count <400,000/microL.[5]

Disposition

Reactive thrombocytosis

  • Disposition is based on underlying disorder.

Autonomous thrombocytosis

  • If asymptomatic, consider outpatient treatment with close follow up.
  • If thrombosis or bleeding complications, should be admitted for stabilization and further work up.

External Links

References

  1. ↑ Schafer, "Thrombocytosis," N Engl J Med 2004;350:1211-9.
  2. ↑ Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.
  3. ↑ Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.
  4. ↑ uptodate
  5. ↑ Bleeker, "Thrombocytosis: Diagnostic Evaluation, Thrombotic Risk Stratification, and Risk-Based Management Strategies," Thrombosis. 2011; 2011: 536062. Published online 2011 Jun 8. doi:10.1155/2011/536062.
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