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16889866
POLICITEMIA VERA
Description
Mind Map on POLICITEMIA VERA, created by Oscar Ulises Durán on 19/02/2019.
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
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Resource summary
POLICITEMIA VERA
Asociada a mutaciones puntuales activadoras de JACK2
Incremento de la producción de eritrocitos, granulocitos y plaquetas
Patogenia
Señalización constitutiva JACK
Células progenitoras transformadas disminuyen los requerimientos de EPO
[EPO] son bajas
Formas secundarias de policitemia: >[EPO]
>Hto; >Viscosidad y aglutinación de la sangre
Trombocitosis y función plaquetaria anormal
Trombosis y hemorragias
97% Mutación del gen JACK2
Sustitución de fenilalanina por valina
Morfología
MO hipercelular
Incremento de progenitores eritrocíticos, granulocíticos y megacariocitos
Clínica
adultos de edad mediana y avanzada
Síntomas asociados a >de eritrocitos, hematocritos y FS
Plétora y cianosis, cefalea, HTA y síntomas GI
Prurito, úlcera péptica, hiperuricemia, gota.
Hemorragias mayores y episodios trombóticos
Leucocitos: 12,000-50,000/mm3; plaquetas>500,000
Tratamiento: Flebotomías
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