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Samenvatting 24-25 Hematologie 3 deel 1 : hematologie

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Samenvatting vak hematologie deel 1 : hematologie Gegeven in 3de jaar MLT

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December 22, 2024
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HEMATOLOGIE 3:
Deel 1 : Hematologie
Lector. Flies Reinoud


Academiejaar
2024 - 2025

,IN HOUD

Herhaling .................................................................................................................................................................. 2

Deel 1 : Afwijkingen van RBC .................................................................................................................................... 3

1. Anemie : inleiding ............................................................................................................................................. 3

2. Afwijkingen van ijzer- en heammetabolisme ................................................................................................... 6

3. Afwijkingen van het DNA-metabolisme ......................................................................................................... 10

4. Hemolytische anemie : Inleiding .................................................................................................................... 10

5. HA : Intracorpusculaire defecten.................................................................................................................... 12

6. HA : Extracorpusculaire defecten ................................................................................................................... 15

7. Hemoglobinopathiën ...................................................................................................................................... 19

8. Thalassemie .................................................................................................................................................... 21

DEEL 2 : Maligne afwijkingen van de WBC ............................................................................................................. 24

1. Inleiding .......................................................................................................................................................... 24

2. Indeling leukemie ........................................................................................................................................... 25

3. Myeloproliferatieve aandoeningen ................................................................................................................ 25

4. Chronische Myeloïde Leukemie ..................................................................................................................... 26

5. Polycythemia vera .......................................................................................................................................... 27

6. Essentiële trombocytemie.............................................................................................................................. 29

7. Myelofibrose (IMF) ......................................................................................................................................... 29

8. Myelodysplastisch syndromen ....................................................................................................................... 30

9. Acute leukemie ............................................................................................................................................... 31

10. Acute Lymfatische Leukemie (ALL) ............................................................................................................... 31

11. Acute Myeloïde Leukemie (AML) ................................................................................................................. 33

12. Lymfoïde Neoplasmata ................................................................................................................................. 34

13. Plasmacelaandoeningen ............................................................................................................................... 38

14. Laboratoriumdiagnostiek Leukemie ............................................................................................................. 40




1
Moons N / Vermeulen A

,HERHALING

REFERENTIEWAARDEN

Man Vrouw Eenheid

RBC 4,3 – 6,0 3,8 – 5,5 Miljoen / µl

HGB 14 – 18 12 – 16 g / dl

HCT 40 – 55 35 – 45 %

MCV 75 – 95 fl

MCH 27 – 32 pg

MCHC 30 – 35 g / dl

RDW < 15 %

WBC 4 000 – 11 000 / µl

TRO 140 000 – 450 000 / µl

RET 0,2 – 2,0 %

Absoluut # RET 60 000 – 100 000 (neonatie 400 000) / µl

RET index 0,4 – 2,22 %


ERYTROPOIESE




2
Moons N / Vermeulen A

, DEE L 1 : AFWIJKIN GEN VAN RBC

Cursus Hematologie : Deel 1: afwijkingen RBC

1. A NE MIE : I NLEIDI NG

1.1 A LGE MEE N
Anemie = bloedarmoede
→ gekenmerkte door daling van Hgb-gehalte

Definitie anemie
• Daling van O2-dragende capaciteit van bloed → komt tot uiting bij verlaagd Hgb-gehalte
• Vermindering van RBC, Hgb en RBC-massa (t.o.v. patiënt zijn basislijn)
22
20
18
Hgb
Hgb-gehalte = parameter voor O2-dragende capaciteit van het bloed 16
14

→ Laag Hgb-gehalte leidt tot weefselhypoxie* 12
10
8
6
Hct = parameter voor bloedvolume en viscositeit 4
2
0




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2m

3m

4m

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2-6


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lw
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6-

12



vo
vo
on
*Weefselhypoxie gevolgen




Ne
• Onvoldoende O2-bevoorrading van omliggende weefsels
• Overconsumptie O2 : verhoogde cardiale en pulmonaire activiteit → Tachycardie
• Verhoogde RBC-productie : door stijging EPO (groeifactor)

Klinische symptomen
• Door weefselhypoxie: bleekheid, moe, syncope, vertigo, dyspnoe (kortademigheid)
• Door compensatie: tachycardie, hartruis, verhoogde RBC-productie (botpijn), A.pectoris

Aan- of afwezigheid klinische symptomen hangt af van 4 factoren
1. Snelheid van ontstaan → Snellere geeft ernstigere symptomen
2. Ernst v anemie → milde anemie : zelden symptomen
3. Leeftijd → Jongeren kunnen beter compenseren
4. Zuurstofdissociatiecurve → anemie geeft verhoogde 2,3-DPG productie


Left shift


Right shift




3
Moons N / Vermeulen A
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