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).


==Differential Diagnosis==
===Autonomous thrombocytosis (AT)<ref>Schafer, "Thrombocytosis," N Engl J Med 2004;350:1211-9.</ref>===
*Reactive thrombocytosis:
*Accounts for 15% of cases
** Acute blood loss
*A primary problem that results from [[myeloproliferative disorders]] or [[myelodysplastic syndrome]]s, such as essential [[thrombocytopenia]] or [[polycythemia vera]].
** Acute hemolytic anemia
*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. 
** Iron deficiency anemia
*There are no diagnostic studies to differentiate RT versus AT.
** Treatment of vitamin B12 deficiency
 
**Rebound effect after thrombocytopenia treatment
==Clinical Features<ref>Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.</ref>==
** Metastatic cancer
''Generally asymptomatic and found on routine lab testing; complications are typically from AT disorders''
** Lymphoma
*Thrombosis (leading cause of morbidity and mortality)
** Rheumatologic disorders
**Arteries (more common)
** IBD
***[[Stroke]], [[TIA]], [[MI]], [[unstable angina]]
** Celiac disease
**Venous system
** Kawasaki disease
***[[DVT]], [[PE]], [[Budd-Chiari syndrome]]
** Nephrotic syndrome
*Serious bleeding (less common)
** POEMS syndrome
**Generally in the nasal and bucchal mucosa and the GI tract
** Thermal burn
*Vasomotor symptoms (due to microvascular disturbances)
** MI
**[[Headache]], lightheadedness, [[syncope]], acral [[paresthesia]], atypical [[chest pain]], livedo reticularis, erythromelalgia, and transient [[visual disturbances]]
** Severe trauma
 
** Acute pancreatitis
==Differential Diagnosis<ref>Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.</ref>==
** Post-surgery, especially splenectomy
===Reactive thrombocytosis===
** CABG
* Acute [[hemorrhage|blood loss]]
** TB
* Acute [[hemolytic anemia]]
** Acute bacterial/viral infections
* Iron deficiency [[anemia]]
** Asplenia
* Treatment of [[vitamin B12 deficiency]]
** Allergic reactions
* Rebound effect after [[thrombocytopenia]] treatment
** Medication reactions: vincristine, epinephrine, glucocorticoids, IL-1B, ATRA, thrombopoietin, LMWH (uptodate)
* Metastatic cancer
*Autonomous thrombocytosis:
* [[Lymphoma]]
** Essential Thrombocytopenia
* Rheumatologic disorders
** Polycythemia Vera
* [[IBD]]
** Mixed myelodysplastic and/or myeloproliferative syndrome
* Celiac disease
*Spurious (false) thrombocytosis:
* [[Kawasaki disease]]
** Mixed cryoglobulinemia
* [[Nephrotic syndrome]]
** Cytoplasmic fragments
* POEMS syndrome
** Bacteremia<ref>Tefferi, "Diagnosis and Clinical Manifestations of Essential Thrombocythemia," UpToDate, Jul. 2017.</ref>
* Thermal [[burn]]
* [[MI]]
* Severe [[trauma]]
* [[Acute pancreatitis]]
* Post-surgery, especially [[splenectomy]]
* CABG
* [[TB]]
* Acute [[bacterial disease|bacterial]]/[[viruses|viral]] infections
* [[asplenic patient|Asplenia]]
* [[Allergic reaction]]
* Medication reactions: vincristine, [[epinephrine]], [[glucocorticoids]], IL-1B, ATRA, thrombopoietin, [[LMWH]]<ref>uptodate</ref>
 
===Autonomous thrombocytosis===
* [[Essential thrombocytosis]]
* [[Polycythemia vera]]
* Mixed [[myelodysplastic syndrome|myelodysplastic]] and/or [[myeloproliferative disorders|myeloproliferative syndrome]]
 
===Spurious (false) thrombocytosis===
* Mixed cryoglobulinemia
* Cytoplasmic fragments
* [[Bacteremia]]


==Evaluation==
==Evaluation==
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==Management==
==Management==
*Reactive thrombocytosis: treat underlying disease.  
===Reactive thrombocytosis===
*Asymptomatic AT:
*Treat underlying disease.  
**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.   
===Autonomous thrombocytosis===
**Second line agents include IFN-alpha, anagrelide, and pipobroman.
====Asymptomatic====
*Thrombosis due to AT:
*81mg [[ASA]] PO daily.
**Anticoagulation with [[LMWH]].
*If high risk  for thrombotic event (>60 yrs, history of thrombosis, or known JAK2 mutation), consider 15mg/kg [[hydroxyurea]] PO daily.  Consult if available.   
**81mg ASA PO daily.
*Second line agents include [[interferon-α]], anagrelide, and pipobroman.
**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.
====Thrombosis due to AT====
**Platelet apheresis if platelet count >800,000/microL.
*Anticoagulation with [[LMWH]].
*Bleeding due to AT:
*81mg [[ASA]] PO daily.
**Discontinue antiplatelet medications.
*Consult heme/onc
**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.
**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>
*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.<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==
*If RT, disposition is based on underlying disorder.   
===Reactive thrombocytosis===
*If asymptomatic AT, consider outpatient treatment with close follow up.   
*Disposition is based on underlying disorder.   
*AT thrombosis or bleeding complications should be admitted for stabilization and further work up.
 
===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==
==External Links==

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.