狠狠撸

Convegno&Internazionale:&
鈥淟鈥檈mo铿乴ia&A&nel&cane鈥�&
Ente&Nazionale&Cino铿乴ia&Italiana&
Verona&1掳&补尘辫;顿颈肠别尘产谤别&补尘辫;2012&补尘辫;

Aspe%&clinici&dell鈥檈mo铿乴ia&A&
nel&cane&
George Lubas
Prof. di Clinica Medica Veterinaria
Dipl. European College Veterinary Internal Medicine
Ass. Dipl. European College Veterinary Clinical Pathology
&

!!Dip.!Scienze!Veterinarie!
Universit脿!degli!Studi!di!Pisa!
1掳&补尘辫;顿颈肠别尘产谤别&补尘辫;2012&补尘辫; Prof.&G.&Lubas& 1&

De铿乶izione&
鈥⑩€� L鈥檈mo铿乴ia&A,&o&emo铿乴ia&classica,&deriva&dalla&
de铿乧ienza&o&assenza&del&faKore&VIII&(F&VIII),&
elemento&essenziale&della&emostasi&secondaria&
Danno Vascolare

vasocostrizione esposizione rilascio di
collagene tromboplastina
tessutale
FvW
cascata
intrinseca cascata
adesione piastrine estrinseca

attivazione e rilascio TROMBINA
piastrine

fibrinogeno

aggregazione trasformazione
piastrinica in fibrina

tappo piastrinico tappo emostatico
1掳&补尘辫;顿颈肠别尘产谤别&补尘辫;2012&补尘辫; primario Prof.&G.&Lubas& definitivo 2&
modulazione dell'emostasi

atti vazi one da contatto
vi vo : col l agene, compl essi A b/A g
vi tr o : super fi ci ar ti fi ci al i , vetr o

fattor i tessutal i
cal l i cr ei na pr ecal l i cr ei na cal l i cr ei na
F XI I a, F I Xa, F Xa
F XI I F XI I a
Ca

F XI F XI a V i a E s t r i n s ec a a l t er n a t i v a

F I Xa F VI I a F VI I
Ca F VI I I :C FI X
F VI I I a + Ca + PF 3
V i a E s t r i n s ec a ( c l a s s i c a )
V i a I n t r i n s ec a
FX F Xa
PT
ACT, aPTT FV F Va + Ca + PF 3

Pr otr ombi na Tr ombi na
Vi a
C om u n e
F i br i nogeno monomer o
F i br i na

Ca F XI I I a F XI I I

pol i mer o
F i br i na

Patogenesi&
鈥⑩€� Gene&posto&sul&cromosoma&X&(legata&al&sesso)&
ad&elevata&potenzialit脿&mutagena&
鈥⑩€� Deriva&da:&
鈥撯€� mutazione&del&gene&(nuovi&casi)&&
鈥撯€� trasmissione&ereditaria&(casi&di&forme&lieviR
moderata)&


Epidemiologia&
鈥⑩€� Prevalenza&ignota&nel&cane&
鈥⑩€� Considerata&la&pi霉&comune&coagulopaUa&
ereditaria&insieme&al&Morbo&di&von&Willebrand&
鈥⑩€� Segnalata&in&molte&razze&pure&(>&40)&e&meUcci&
鈥⑩€� Nell鈥檜omo&incidenza&di&1/30.000&maschi&


Due&Upi&
principali&di&
alterazione&
dell鈥檈mostasi&
(modi铿乧ata&da&Willard&et&
al.,&1999)&


Sintomatologia&clinica&
鈥⑩€� Si&riconoscono&tre&forme&cliniche&in&relazione&
all鈥檃%vit脿&del&FVIII:&
鈥撯€� Lievi&FVIII&&5R20%&
鈥撯€� Moderata&FVIII&1R5%&
鈥撯€� Gravi&FVIII&<1%&
鈥⑩€� Il&sanguinamento&clinico&delle&tre&forme&pu貌&non&
essere&correlato&alla&quanUt脿&del&FVIII&
鈥⑩€� La&gravit脿&del&sanguinamento&clinico&pu貌&essere&
correlato&alla&taglia&e&all鈥檃%vit脿&铿乻ica&del&cane&

Forme&cliniche&
鈥⑩€� Cuccioli&
鈥撯€� Sanguinamento&persistente&ombelico&
鈥撯€� Ematomi&cutanei&
鈥撯€� Emorragie&gengivali&alla&denUzione&permanente&
鈥⑩€� Giovani,&adulU&
鈥撯€� Zoppie&intermiKenU&e&migranU&da&emartri&
鈥撯€� Ematomi&muscolari&
鈥撯€� Emorragie&perioculari&e&oculari&
鈥撯€� Emorragie&mediasUniche,&emotorace&e&
retroperitoneale&(铿乶o&a&morte)&
鈥撯€� Emorragie&nel&midollo&spinale&e&SNC&


Emorragia&ombelicale&persistente&in&un&cucciolo&
emo铿乴ico&(LiKlewood&JD,&2000)&


Ematomi&cutanei&di铿€usi&nel&cucciolo&


Emorragia&gengivale&al&cambio&
denUzione&(LiKlewood&JD,&2000)&

Emorragia&del&cavo&orale&

Ematomi&cutanei&


Ematomi&cutanei&ed&arUcolari&


Ematomi&muscolari&


Radiologia&e&Emartri&


Postumi&di&ematomi&
Postumi&di&ematomi&
intraossei&&
intramuscolari&


Pubblicazioni&emo铿乴ia&A&nel&passato&


SiU&web&in&collaborazione&


Pubblicazioni&
recenU&
sull鈥檈mo铿乴ia&A&


FACTOR VIII LEVELS IN A GROUP OF BELGIAN SHEPHERD MALINOIS DOGS BREED
IN ITALY
Lubas G. , Gavazza A. 1, Caldin M 2
1

1- Dept. of Veterinary Clinic, University of Pisa; 2- San Marco Veterinary Laboratory, Padua,
Italy

Occurrence of Hemophilia A in the Belgian Shepherd Malinois (BSM) dog has been recently
described. This is the most common and severe inherited canine coagulopathy. Diagnosis is based
on clinical signs, prolonged aPTT, and reduction in factor VIII activity. The study aim was to assess
the level of FVIII in BSM dogs in Italy.
Fifty-four studbook registered BSMs have been investigated (34 males, 20 females), in which
Hemophilia A was a possible condition. For each BSM, the genealogical data and, whenever
possible, the history and other clinical data were collected. All BSMs were tested for FVIII
concentration using two reference labs. When possible, the FvW concentration in females was also
assessed (carrier FVIII/FvW <0.60). Most of the data are displayed in a free accessible web site
(www.malinemo.net).
Ten male dogs had low level of FVIII (range 5-21%, consistent with Hemophilia A), four females
showed FVIII between 28-55% (carrier range), and 21 dogs resulted with FVIII level >70%.
Nineteen dogs showed FVIII level at the borderline either for carrier or clear range.
The present work supports the evidence of Hemophilia A in BSM dogs, with a relatively high
frequency. Unfortunately, the level of FVIII alone does not allow a clear set between carrier and
clear dogs. Only the complete clinical examination, together with laboratory data, should rule-out
concurrent disorders (i.e. liver failure or acquired coagulopathy). Further studies on factor VIII gene
mutations using offspring to correct identify the Hemophilia A in BSM dogs are in progress.

Cucciolo&cane&corso&(FvW=62%,&FVIII=3%)&


Convegno&Internazionale:&
鈥淟鈥檈mo铿乴ia&A&nel&cane鈥�&
Ente&Nazionale&Cino铿乴ia&Italiana&
Verona&1掳&补尘辫;顿颈肠别尘产谤别&补尘辫;2012&补尘辫;

Aspe%&clinici&dell鈥檈mo铿乴ia&A&
nel&cane&
George Lubas
Prof. di Clinica Medica Veterinaria
Dipl. European College Veterinary Internal Medicine
Ass. Dipl. European College Veterinary Clinical Pathology
&

!!Dip.!Scienze!Veterinarie!
Universit脿!degli!Studi!di!Pisa!

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