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son aquellas que afectan la producción de los elementos formes de la
sangre como: los glóbulos rojos, glóbulos blancos, la hemoglobina,
las proteínas plasmáticas, el mecanismo de coagulación
(hemostasia), causando serios problemas a nivel sistémico.
TRASTORNOS HEMATOLOGICOS EN NIÑOS
EPISTAXIS
salida de sangre al exterior por vía anterior
o posterior, de origen endonasal, retronasal
o extranasal, causada por la ruptura de los
elementos vasculares que garantizan la
irrigación de las fosas nasales, senos
paranasales y la nasofaringe.
Trastornos de la Coagulación
Déficit de coagulación o trastornos de la
misma incluyen la Enfermedad de Von
Willebrand, púrpura trombocitopénica
idiopática (PTI), trombocitopenia, hemofilia,
deficiencias de factores, las deficiencias de
vitaminas (A, D, C, E, o K), leucemia y otras
coagulopatías congénitas y adquiridas.
la enfermedad de Von Willebrand, se manifiesta por sangrado espontáneo de
las mucosas. Se caracteriza por un tiempo de sangrado prolongado, una
deficiencia de factor VIII y un deterioro de adhesividad de las plaquetas con un
recuento normal de las mismas.
Las enfermedades vasculares en los niños incluyen la Telangiectasia
Hemorrágica Hereditaria (THH), alteraciones del colágeno y otros tipos de
vasculitis. Aquí se excluyen los hemangiomas y las malformaciones
vasculares como aneurismas.
La hemofilia presentan hemorragias tisulares (p. ej., hemartrosis, hematomas musculares,
hemorragia retroperitoneal). La hemorragia puede ser inmediata o producirse lentamente, lo que
depende del grado de traumatismo y de la concentración plasmática de factor VIII o IX.
.
LEUCEMIAS
Es la proliferacion neoplasica de celulas
hematopoyeticas en una estirpe celular con
posterior proliferacion y expansion.
.
se origina en las células que
normalmente madurarían hacia
los diferentes tipos de células
sanguíneas.
la leucemia se origina en formas tempranas
de glóbulos blancos, pero algunas leucemias
comienzan en otros tipos de células
sanguíneas
AGUDA
crecimiento rápido
mieloide
linfoide
CRONICA
crecimiento más lento
mieloide
linfoide
Leucemia linfocítica (linfoblástica)
aguda (LLA).
Este es el tipo de leucemia más común en los
niños Se inicia a partir de los linfoblastos, las
células que forman los linfocitos en la médula
ósea.
Se inicia a partir de los mieloblastos o células
mieloides que forman muchos glóbulos blancos,
además de glóbulos rojos y plaquetas.
las células de la línea mieloide (mieloblastos) proliferan
de forma anormal invadiendo progresivamente la
médula ósea e interfiriendo la producción de células
normales de la sangre, lo que origina insuficiencia
medular e infiltra tejidos extramedulares.
las células cancerosas desplazan las células
sanguíneas sanas, lo cual puede causar
fiebre, fatiga, formación de moretones con
facilidad, sangrado, infecciones y otros
problemas
Leucemia mielógena (mieloide, mielocítica,
no linfocítica) aguda (LMA).
tienen demasiados glóbulos blancos inmaduros
(blastos) en su médula ósea. Estas células no
funcionan con normalidad. Ellas desplazan a los
glóbulos blancos, los glóbulos rojos y las plaquetas
normales.
el cuerpo tiene mayor dificultad para combatir
infecciones, la piel se vuelve pálida y el paciente
desarrolla problemas de sangrado.
Leucemia mielógena
crónica (LMC).
la progresión es insidiosa, con un estadio "benigno"
asintomático (malestar general, anorexia, descenso de
peso) que finalmente da lugar a estadios acelerados o
blásticos con signos más ominosos.

.
Los signos y los síntomas pueden estar ausentes o
pueden observarse adenopatías, esplenomegalia,
hepatomegalia, fatiga, fiebre, sudoración nocturna,
pérdida de peso no intencional y saciedad temprana
.
a LMC es una leucemia crónica. Esto significa que se
desarrolla lentamente con el paso del tiempo. Por eso, pueden
pasar semanas o meses hasta que los niños desarrollen
síntomas
Al igual que la LMA, se inicia a partir de los mieloblastos
o células mieloides que forman muchos glóbulos
blancos, además de glóbulos rojos y plaquetas.
acumulación progresiva de linfocitos B malignos
fenotípicamente maduros. Los sitios primarios de la
enfermedad incluyen la sangre periférica, la médula
ósea, el bazo y los ganglios linfáticos.
La leucemia linfocítica crónica ocurre cuando hay cambios
en el material genético (ADN) de las células de la médula
ósea. No se conoce la causa de estos cambios genéticos.
Leucemia linfocítica
crónica (LLC).
CAUSAS SINTOMAS
Se han encontrado cambios
(mutaciones) en los genes de las
células de la médula ósea. Estos
cambios pueden ocurrir temprano en
la vida de un niño o incluso antes del
nacimiento
Los síntomas pueden ser levemente distintos en cada niño. Estos
pueden incluir:
La púrpura es producida por la extravasación de células sanguíneas a la piel
y/o mucosas, dando origen a lesiones de coloración purpúrea que no
desaparecen a la vitropresión.
Se clasifican de acuerdo con su localización.
PURPURAS
Púrpuras superficiales: se denominan petequias
las lesiones menores de 2 mm y equimosis las
de mayor de 1 cm. . Las de tamaño intermedio
constituyen la púrpura propiamente dicha.
Púrpuras profundas o hematomas: cuando
invade los distintos estratos cutáneos
Etiopatogenia
Puede producirse por alteración de alguno de los tres
componentes de la hemostasia: plaquetas, factores de la
coagulación o lesiones de la pared vascular (vasculitis).
púrpuras trombopénicas o
plaquetopenias
se produce una disminución aislada
del recuento plaquetario a valores
<100-150.000/ μl
Producidas por descenso de
la producción plaquetaria,
que puede ser de origen
congénito o adquirido.
Por un aumento de la
destrucción de plaquetas
de causa inmunitaria o no
inmunitario
Por alteración de la
distribución plaquetar con
secuestro de plaquetas en
un bazo aumentado de
tamaño o en otros órganos
Las púrpuras trombopáticas o
plaquetopatía
son cuadros más infrecuentes,
debidos a alteraciones de la
función plaquetaria
es un tiempo de hemorragia/tiempo de obturación
(TO) alargado, con un número de plaquetas
normal o discretamente disminuido
La púrpura de Schönlein-
Henoch (PSH).
es una enfermedad en la
que los vasos sanguíneos
pequeños se hinchan e
irritan, a esta afección se le
llama vasculitis.
dolor en las articulaciones,
dolor abdominal o
problemas renales.
sarpullido llamado púrpura
palpable que se presenta
como manchas rojizas
oscuras en los pies, las
piernas y las nalgas.
Se aconseja reposo en
cama los primeros días. Los
AINE se emplean para
aliviar las molestias
articulares.
Púrpura trombocitopénica inmune (idiopática) en
niños
es un trastorno de la sangre que provoca una disminución
en el número de plaquetas en la sangre.
La PTI puede ser aguda o
crónica:
Púrpura trombocitopénica aguda. Es la más común en
niños pequeños (de entre 2 y 6 años de edad).
Púrpura trombocitopénica crónica. Este trastorno puede
comenzar a cualquier edad. Los síntomas duran por lo
menos 12 meses
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leucemias 2.pptx

  • 1. son aquellas que afectan la producción de los elementos formes de la sangre como: los glóbulos rojos, glóbulos blancos, la hemoglobina, las proteínas plasmáticas, el mecanismo de coagulación (hemostasia), causando serios problemas a nivel sistémico. TRASTORNOS HEMATOLOGICOS EN NIÑOS
  • 2. EPISTAXIS salida de sangre al exterior por vía anterior o posterior, de origen endonasal, retronasal o extranasal, causada por la ruptura de los elementos vasculares que garantizan la irrigación de las fosas nasales, senos paranasales y la nasofaringe. Trastornos de la Coagulación Déficit de coagulación o trastornos de la misma incluyen la Enfermedad de Von Willebrand, púrpura trombocitopénica idiopática (PTI), trombocitopenia, hemofilia, deficiencias de factores, las deficiencias de vitaminas (A, D, C, E, o K), leucemia y otras coagulopatías congénitas y adquiridas.
  • 3. la enfermedad de Von Willebrand, se manifiesta por sangrado espontáneo de las mucosas. Se caracteriza por un tiempo de sangrado prolongado, una deficiencia de factor VIII y un deterioro de adhesividad de las plaquetas con un recuento normal de las mismas. Las enfermedades vasculares en los niños incluyen la Telangiectasia Hemorrágica Hereditaria (THH), alteraciones del colágeno y otros tipos de vasculitis. Aquí se excluyen los hemangiomas y las malformaciones vasculares como aneurismas. La hemofilia presentan hemorragias tisulares (p. ej., hemartrosis, hematomas musculares, hemorragia retroperitoneal). La hemorragia puede ser inmediata o producirse lentamente, lo que depende del grado de traumatismo y de la concentración plasmática de factor VIII o IX. .
  • 4. LEUCEMIAS Es la proliferacion neoplasica de celulas hematopoyeticas en una estirpe celular con posterior proliferacion y expansion. . se origina en las células que normalmente madurarían hacia los diferentes tipos de células sanguíneas. la leucemia se origina en formas tempranas de glóbulos blancos, pero algunas leucemias comienzan en otros tipos de células sanguíneas
  • 6. Leucemia linfocítica (linfoblástica) aguda (LLA). Este es el tipo de leucemia más común en los niños Se inicia a partir de los linfoblastos, las células que forman los linfocitos en la médula ósea. Se inicia a partir de los mieloblastos o células mieloides que forman muchos glóbulos blancos, además de glóbulos rojos y plaquetas. las células de la línea mieloide (mieloblastos) proliferan de forma anormal invadiendo progresivamente la médula ósea e interfiriendo la producción de células normales de la sangre, lo que origina insuficiencia medular e infiltra tejidos extramedulares. las células cancerosas desplazan las células sanguíneas sanas, lo cual puede causar fiebre, fatiga, formación de moretones con facilidad, sangrado, infecciones y otros problemas Leucemia mielógena (mieloide, mielocítica, no linfocítica) aguda (LMA). tienen demasiados glóbulos blancos inmaduros (blastos) en su médula ósea. Estas células no funcionan con normalidad. Ellas desplazan a los glóbulos blancos, los glóbulos rojos y las plaquetas normales. el cuerpo tiene mayor dificultad para combatir infecciones, la piel se vuelve pálida y el paciente desarrolla problemas de sangrado.
  • 7. Leucemia mielógena crónica (LMC). la progresión es insidiosa, con un estadio "benigno" asintomático (malestar general, anorexia, descenso de peso) que finalmente da lugar a estadios acelerados o blásticos con signos más ominosos.  . Los signos y los síntomas pueden estar ausentes o pueden observarse adenopatías, esplenomegalia, hepatomegalia, fatiga, fiebre, sudoración nocturna, pérdida de peso no intencional y saciedad temprana . a LMC es una leucemia crónica. Esto significa que se desarrolla lentamente con el paso del tiempo. Por eso, pueden pasar semanas o meses hasta que los niños desarrollen síntomas Al igual que la LMA, se inicia a partir de los mieloblastos o células mieloides que forman muchos glóbulos blancos, además de glóbulos rojos y plaquetas. acumulación progresiva de linfocitos B malignos fenotípicamente maduros. Los sitios primarios de la enfermedad incluyen la sangre periférica, la médula ósea, el bazo y los ganglios linfáticos. La leucemia linfocítica crónica ocurre cuando hay cambios en el material genético (ADN) de las células de la médula ósea. No se conoce la causa de estos cambios genéticos. Leucemia linfocítica crónica (LLC).
  • 8. CAUSAS SINTOMAS Se han encontrado cambios (mutaciones) en los genes de las células de la médula ósea. Estos cambios pueden ocurrir temprano en la vida de un niño o incluso antes del nacimiento Los síntomas pueden ser levemente distintos en cada niño. Estos pueden incluir:
  • 9. La púrpura es producida por la extravasación de células sanguíneas a la piel y/o mucosas, dando origen a lesiones de coloración purpúrea que no desaparecen a la vitropresión. Se clasifican de acuerdo con su localización. PURPURAS Púrpuras superficiales: se denominan petequias las lesiones menores de 2 mm y equimosis las de mayor de 1 cm. . Las de tamaño intermedio constituyen la púrpura propiamente dicha. Púrpuras profundas o hematomas: cuando invade los distintos estratos cutáneos
  • 10. Etiopatogenia Puede producirse por alteración de alguno de los tres componentes de la hemostasia: plaquetas, factores de la coagulación o lesiones de la pared vascular (vasculitis). púrpuras trombopénicas o plaquetopenias se produce una disminución aislada del recuento plaquetario a valores <100-150.000/ μl Producidas por descenso de la producción plaquetaria, que puede ser de origen congénito o adquirido. Por un aumento de la destrucción de plaquetas de causa inmunitaria o no inmunitario Por alteración de la distribución plaquetar con secuestro de plaquetas en un bazo aumentado de tamaño o en otros órganos Las púrpuras trombopáticas o plaquetopatía son cuadros más infrecuentes, debidos a alteraciones de la función plaquetaria es un tiempo de hemorragia/tiempo de obturación (TO) alargado, con un número de plaquetas normal o discretamente disminuido
  • 11. La púrpura de Schönlein- Henoch (PSH). es una enfermedad en la que los vasos sanguíneos pequeños se hinchan e irritan, a esta afección se le llama vasculitis. dolor en las articulaciones, dolor abdominal o problemas renales. sarpullido llamado púrpura palpable que se presenta como manchas rojizas oscuras en los pies, las piernas y las nalgas. Se aconseja reposo en cama los primeros días. Los AINE se emplean para aliviar las molestias articulares. Púrpura trombocitopénica inmune (idiopática) en niños es un trastorno de la sangre que provoca una disminución en el número de plaquetas en la sangre. La PTI puede ser aguda o crónica: Púrpura trombocitopénica aguda. Es la más común en niños pequeños (de entre 2 y 6 años de edad). Púrpura trombocitopénica crónica. Este trastorno puede comenzar a cualquier edad. Los síntomas duran por lo menos 12 meses
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