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16908641
TROMBOCITOSIS ESENCIAL
Description
Mind Map on TROMBOCITOSIS ESENCIAL, created by Oscar Ulises Durán on 20/02/2019.
Mind Map by
Oscar Ulises Durán
, updated more than 1 year ago
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Created by
Oscar Ulises Durán
over 5 years ago
20
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Resource summary
TROMBOCITOSIS ESENCIAL
Asociado a mutaciones
Puntuales: Activadoras JACK2
MLP
Normalmente activado por EPO
Calreticulina
Aumentan señales JACK-STAT
Clínica
Elevación de plaquetas
Ausencia de policitemia ni fibrosis medular
Deficiencia de hierro
Disfunción de plaquetas
Trombosis y hemorragias
TrVP, y de venas porta y hepática, infarto de miocardio, eritromelalgia
Patogenia
Señalización constitutiva JACK O MPL
Progenitores independientes de trombopoyetina
Mutación JACK2
Igual que en PCV
Celularidad de MO
Incremento leve
++Megacariocitos; Anormalmente grandes
Delicadas fibrillas de reticulina
Frotis periféricos
Plaquetas anormalmente grandes
Leucocitosis
Hematopoyesis extrañedular
Organomegalia leve
Epidemiología
>Después de 60a
También en adultos jóvenes
Pronóstico
Supervivencia 12-15 años
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