Fibrinogen

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Fibrinogen Advisor Thanusak
Tatu
By Rutai Yaicheun
4211065 Sukanya Yoadsang
4211083 Supinya Iamsawat
4211089

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FIBRINOGEN
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Properties of fibrinogen Clotting pathway
Intrinsic , Extrinsic , Common pathway
Molecular mass 340,000 Da Site of
production liver Plasma concentration 200-400
mg/dl Half life 3-5 days Minimum hemostatic
level 50-100 mg Storage stable Active form
Protein substrate Subunit formula
abg
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Structure
Fibrinogen ???? dimeric molecule
?????????? polypeptides chain 3
????????????? disulfide bond
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(No Transcript)
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FUNCTION
OF
FIBRINOGEN
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1.Bridges platelet during platelet aggregation

????????? fibrinogen specific receptor ??
gpIIb/IIIa ??? activated platelets
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2. ??????????????????????? stable fibrin
????????????????????? Thrombin ??? factor XIII
Fibrinogen

Thrombin


Fibrin monomer


XII

Fibrin
polymer
XIIa



Fibrinopeptide release
Hydogen bonding
Transamidase bonding
Cross-linked fibrin
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Thrombin ??????????????? arginine ??? glycine
?????? N-terminal ?????????fibrinopeptide A
???fibrinopeptide B ????? ????????? fibrin
monomer
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Factor XIII ????????? covalent bond
??????? glutamine ??? lysine ???fibrin
??????????? ??????????????? stable fibrin
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Fibrinogen abnormalities ???????? 2 ??????
1.Quantitative abnormalities Afibrinogenemia
(????????????? fibrinogen) Hypofibrinogenemia
(?????????? fibrinogen ???????? 100 mg/dl
???????? ) 2.Qualitative abnormalities
Dysfibronogenemia ( ?????????????????
fibrinogen????????? ??????????????????)
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Congenital Afibrinogenemia ???
Hypofibrinogenemia Afibrinogenemia ??????????????
??????????????????????????????????????????????
?????????????????????????????????????
???? ????????????
????????????? Hemophilia ??? Hypofibrinogenemia ?
????????????? ?? 3 ???????? ????????????
???????????????
?????????????? Congenital Dysfibrinogenemia ?????
????????? ???????????????????????????????
???????????????????
??????????????????
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DIFFERENTIAL DIAGNOSIS OF FIBRINOGEN
DISORDER TEST S
AFIBRINOGENEMIA HYPOFIBRINOGENEMIA
DYSFIBRINOGENEMIA Bleeding time
P N
N Prothrombin time
P P
P APTT
P P
P Thrombin time
P P
P Fibrinogen level 10
mg/dl 20-100 mg/dl 10
mg/dl Platelet aggregation Abnormal
N N
Fibrinolytic test
N N
N
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Case study
A Thai boy ,ages7 years old , was
admitted due to an oozing from the wound
after his circumcision. PE An active
boy with normal growth and no jaundice
BT 37.2 oC .BP 110/75mmHg Hearth and
lung Clear Liver Spleen
Not palpable
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Blood examination CBC Hb 11.0 g (normal
10-15 g) Hct 34 ( normal 32-44 )
WBC 5,600 / cu.mm.(normal 5,000-10,000/cu.mm.)
PMN 65 (normal 35-65 ) Lymphocytes 35 (
normal 25-75 ) Platelet on smear Adequate
with normal shape and size and
stain Platelet count 328,000 /
cu.mm.( normal 140,000-440,00
cu.mm.) RBC morphology With normal limit
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RBC morphology With normal limit MCV
85 fl (normal 69 90 fl ) MCH
25 pg ( normal 20 26 pg) MCHC 32 g (
normal 31-34 g )
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Bleeding time (Ivy s method) 3 min (Normal
0.5 - 6.0 ) VCT 30 min ( Normal 9 15
min) Coagulogram PT 23 sec ( Normal 12
15 sec ), Control 13.0 sec APTT
50 sec ( Normal 28 40 sec ), Control
25 sec TT 45 ( Normal 7 - 10 sec ),
Control 8 sec Fibrinogen level 10
mg/dl ( Normal 200- 400 mg/dl )
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Bleeding time (Ivy s method) 3 min
(Normal 0.5 - 6.0 ) ??????????????????????????
??????????????? ????????????? ???????????????????
?????????? Bleeding time Normal
Prolong Thrombocytopenia
platelet dysfunction ??? ??????? C
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Prothrombin time ( PT ) 23 sec ( normal
12-15 sec , control 13.0 sec ) ???????????
activity ???????????????????????? extrinsic
pathway ?????????????? factor V,VII,X,II ???
fibrinogen prolong factor V,VII.X
activity lt 20-25 factor II lt 10
fibrinogen lt 1 g/l heparin therapy
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Activated partial thromboplastin time (APTT)
50 sec ( normal 28-40 sec,control 25 sec )
?????????????????????????? Intrinsic pathway
Prolong factor XII , XI , IX , VIII, X
,V activity lt 25 prothrombin lt 10
fibrinogen ???????? lt 1 g/l
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Thrombin time ( TT) 45 sec ( Normal 7-10 sec
, control 8 sec ) ??????????????????????????
fibrinogen ?????????? ??????????????? Fibrinogen
level 10 mg/dl ( normal 200-400 mg/dl )
Hypofibrinogenemia Abruptio placentae
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Coagulogram PT, APTT, TT Prolong
Cause Factor deficiency ( I
) Severe liver disease not palpable
DIC Hypercoagulability
Hyperfibrinolysis FDP
??????????? Thrombocytopenia
Hypofibrinogenemia Dysfibrinogenemia
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Diagnosis ???????????????????????????????
?????????????? ??????????? Congenital
hypofibrinogenemia ????????? ?????
fibrinogen 10 mg/dl Bleeding time
???? PT, APTT, TT prolong
?????????????? Liver cirrhosis
DIC Hypofibrinogenemia
Dysfibrinogenemis
?????????????????????? ??? Liver cirrhosis
??????? Hypercoaglulation ,Hyperfibrinolysis,
Thrombocytopenia ??? DIC