Diferencia entre revisiones de «Essential thrombocytosis»
(Created page with "==Background== *Rare, chronic myeloproliferative neoplasm *Abnormal proliferation is seen in megakaryocytic line only *Typically in elderly *Generally asymptomatic ==Clinical...") |
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==Background== | ==Background== | ||
*Rare, chronic myeloproliferative neoplasm | *Rare, chronic [[myeloproliferative disorders|myeloproliferative neoplasm]] | ||
*Abnormal proliferation is seen in megakaryocytic line only | *Abnormal proliferation is seen in megakaryocytic line only | ||
*Typically in elderly | *Typically in elderly | ||
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==Clinical Features== | ==Clinical Features== | ||
* | *[[Leukocytosis]] | ||
* | *[[Anemia]] | ||
* | *Splenomegaly | ||
*Clotting or [[coagulopathy|bleeding]] | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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*[[Chronic myelogenous leukemia]] (CML) | *[[Chronic myelogenous leukemia]] (CML) | ||
*[[Polycythemia vera]] (PV) | *[[Polycythemia vera]] (PV) | ||
===Reactive thrombocytosis=== | |||
* Acute [[hemorrhage|blood loss]] | |||
* Acute [[hemolytic anemia]] | |||
* Iron deficiency [[anemia]] | |||
* Treatment of [[vitamin B12 deficiency]] | |||
* Rebound effect after [[thrombocytopenia]] treatment | |||
* Metastatic cancer | |||
* [[Lymphoma]] | |||
* Rheumatologic disorders | |||
* [[IBD]] | |||
* Celiac disease | |||
* [[Kawasaki disease]] | |||
* [[Nephrotic syndrome]] | |||
* POEMS syndrome | |||
* 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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===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>=== | ===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>=== | ||
Per the WHO guidelines, diagnosis of ET requires all of the following: | |||
{| class="wikitable" | {| class="wikitable" | ||
|- | |- | ||
! | ! Major criteria | ||
|- | |- | ||
| Thrombocytosis ≥ 450 x 10<sup>9</sup>L | | Thrombocytosis ≥ 450 x 10<sup>9</sup>L | ||
|- | |||
| Megakaryocyte proliferation with little to no granulocyte or erythroid proliferation | | Megakaryocyte proliferation with little to no granulocyte or erythroid proliferation | ||
|- | |- | ||
| Not meeting criteria for CML, PV, PMF, MDS or other myeloid neoplasm | | Not meeting criteria for CML, PV, PMF, MDS or other myeloid neoplasm | ||
|- | |- | ||
| | | No evidence of reactive thrombocytosis or presence of JAK2V617F or other clonal marker | ||
|} | |} | ||
==Management== | ==Management== | ||
*ASA 81mg | *[[ASA]] 81mg | ||
*Myelosuppressive agents (hydroxyurea, | *Myelosuppressive agents ([[hydroxyurea]], Interferon-α) | ||
*Anagrelide | |||
==Disposition== | |||
==Also See== | ==Also See== | ||
Revisión actual - 00:27 1 oct 2019
Background
- Rare, chronic myeloproliferative neoplasm
- Abnormal proliferation is seen in megakaryocytic line only
- Typically in elderly
- Generally asymptomatic
Clinical Features
- Leukocytosis
- Anemia
- Splenomegaly
- Clotting or bleeding
Differential Diagnosis
Reactive thrombocytosis
- Acute blood loss
- Acute hemolytic anemia
- Iron deficiency anemia
- Treatment of vitamin B12 deficiency
- Rebound effect after thrombocytopenia treatment
- Metastatic cancer
- Lymphoma
- Rheumatologic disorders
- IBD
- Celiac disease
- Kawasaki disease
- Nephrotic syndrome
- POEMS syndrome
- Thermal burn
- MI
- Severe trauma
- Acute pancreatitis
- Post-surgery, especially splenectomy
- CABG
- TB
- Acute bacterial/viral infections
- Asplenia
- Allergic reaction
- Medication reactions: vincristine, epinephrine, glucocorticoids, IL-1B, ATRA, thrombopoietin, LMWH[1]
Autonomous thrombocytosis
Spurious (false) thrombocytosis
- Mixed cryoglobulinemia
- Cytoplasmic fragments
- Bacteremia
Evaluation
Workup
- CBC
- Chem 7
- ESR
- Bone marrow biopsy sometimes needed
Evaluation[2]
Per the WHO guidelines, diagnosis of ET requires all of the following:
| Major criteria |
|---|
| Thrombocytosis ≥ 450 x 109L |
| Megakaryocyte proliferation with little to no granulocyte or erythroid proliferation |
| Not meeting criteria for CML, PV, PMF, MDS or other myeloid neoplasm |
| No evidence of reactive thrombocytosis or presence of JAK2V617F or other clonal marker |
Management
- ASA 81mg
- Myelosuppressive agents (hydroxyurea, Interferon-α)
- Anagrelide