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Samenvatting Myeloproliferatieve aandoeningen

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Dit is een samenvatting met notities van de les van Dr. Louagie in het tweede jaar MLT voor het vak hematologie. Aan de samenvatting zijn verduidelijkende figuren toegevoegd. Dit onderdeel gaat over de myeloproliferatieve aandoeningen.

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  • May 22, 2019
  • 6
  • 2017/2018
  • Summary
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Myeloproliferatieve
aandoeningen
Soorten
1. Polycythaemia rubra vera (PRV) = M. Vaquez: + #RBC
2. Chronische myeloïde leukemie (CML): + #WBC
3. Essensiële trombocytose (ET): + #TC
4. Idiopatische myelofibrose (IMF)
5. Chronische eosinofiele leukemie (CEL)
6. Chronische neutrofiele leukemie (CNL)

Andere voorkomens
1. Niet altijd bij +#RBC: M. Vaquez, anders:
a. EPO producerende tumoren
b. Hypoxie
i. Grote hoogtes
ii. Longlijders
iii. rokers
2. Bij infecties




Bij hypoxie: -- O2 transport

Bloed komt in BV en bezit
‘metertje’: geeft signaal dat er
te weinig O2 in bloed zit aan
nier

Nier: EPO aanmaak

Wanneer bloed met
voldoende O2 in BV: stop
aanmaak EPO

, Indeling
2008




2016
- Mastocytose is een aparte entiteit
- Myeloied/lymfoied neoplasie met eosinofilie en rearrangement van PDGFRA, PDGFRB of
PGFR1 of PCM1-Jak2: aparte entiteit
- CEL blijft bij de MPD

CML
Variaties
- CML in chronische fase
- CML in acceleratie
- CML in blastencrisis
Karyotype




- Translocatie: ~t(9,22) = uitwisseling tussen chromosoom 9 en 22  Philadelphia
Chromosoom
- BCR/ABL = eiwit: tyrosine kinase  woekering WBC

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