apraga/org - Change CXHSL4PH3O6XZZFE4HAON6OPGX2AT4EDKWLCJ5PNL5JERYX2J6FAC

Willebrand notes

Created by Alexis Praga on February 12, 2024

CXHSL4PH3O6XZZFE4HAON6OPGX2AT4EDKWLCJ5PNL5JERYX2J6FAC

Dependencies

In channels

main

Change contents

File addition: .gitignore (----------)
[3.2112]
```
site.tar.gz
site
```

File addition: willebrand.svg (----------)

[3.8342]

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<title>vWFpp&#45;&gt;Type 3</title>
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<title>FVIII/ag vWF</title>
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<!-- Type 2&#45;&gt;2A\nsi HPM\nabsents -->
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<title>2B sinon</title>
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<g id="edge13" class="edge">
<title>Type 2&#45;&gt;2B sinon</title>
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File addition: willebrand.dot (----------)

[3.8342]

digraph {
     "Ag vWF" -> vWFpp [label="indétectable"];
    vWFpp ->  "Type 3" [label="absent"];  
    vWFpp -> "Type 1 sévère" [label="présent"] ;
    "Ag vWF" -> "activité/Ag vWF"[label="sinon"];
    "activité/Ag vWF" -> "FVIII/ag vWF"[label="> 0.7"];
    "FVIII/ag vWF" -> "Type 1 et 2M" [label="> 0.7"];
    "FVIII/ag vWF" -> "vWF:FVIIIb"  [label="< 0.7"];
     "vWF:FVIIIb" -> "2N"[label="diminuée"]; 
     "vWF:FVIIIb" -> "Hémophilie A"[label="normal"]; 
    "activité/Ag vWF" -> "Type 2"[label="< 0.7"];
    "Type 2" -> { "2B\nPT-VWD\nsi RIPA\naugmenté", "2A\nsi HPM\nabsents"; "2B sinon";}
    // "diminué" -> "type 2N" ; 
    // "normal" -> "Hémophilie A";
    // "multimère" -> {"HPM absent" -> "Type 2A";
    // "HPM présents" -> "Type 2M3 }
    // }
}

Insertion in medecine/hematologie.md at line 42 [4.426]

[4.1689]


# Cytométrie en flux
Identification des cellules hématopoïetique par glycoprotéines (surface/cytoplasme) = antigènes de différentiation
- anticorps monoclonaux + fluorochrome
- regroupés en cluster de différenciation (CD) -> sous-populations
Sur sang (EDTA), fluide (tube sec), tissu (enzyme)
Principe : cellule "s'allume" (via fluorochrome) en passant devant un laser
Indication en hémato: hémopathies malignes
- leucémie, lymphome
- leucocytose (hyperéosinophilie, infection (neutro, mono)), neutropénie, LGL
- anémie : HPN, sphérocytose, GF foetaux
- plaquettes : numération, déficit

Insertion in medecine/hematologie.md at line 208 [4.426]

[2.1556]


Type 1 = déficit quantitatif
Type 2 = déficit qualitatif (2A = déficit vWF, 2B = gain de fonction avec déplétion multimere de haut poids moléculaire (HMW), 2M = qualitatif, 2N seulement binding FVIII)
Type 3 = quasi-absence vWF

Insertion in medecine/hematologie.md at line 231 [4.426]
[2.2097]
[2.2097]
```
![](../../images/hematologie/willebrand.svg)
```

Deletion in medecine/hematologie.md at line 233 [4.426]

B:BD[2.2098] → [2.2098:2528]

∅:D[2.2528] → [5.221:222]

B:BD[4.6682] → [5.221:222]

"Ag vWF" -> indétectable -> vWFpp -> { absent -> "Type 3"; present -> "Type 1 sévère" }
"Ag vWF" -> "activité/ag vWF" -> "> 0.7" -> "FVIII/ag vWF" -> 
{ "> 0.7" -> "Type 1 et 2M";
"< 0.7" -> "vWF:FVIIIB" -> { "diminué -> "type 2N" ; "normal" -> "Hémophilie A"}
"activité/ag vWF" -> "< 0.7" -> "type 2" -> { RIPA augmenté -> "type 2B/PT-VWD"  ;
"multimère" -> {"HPM absent" -> "Type 2A";
"HPM présents" -> "Type 2M3 }
}