Hereditary and acquired coagulation disorders are caused by deficiencies in clotting factors that lead to defects in normal clot formation. Hereditary disorders include hemophilia A/classical hemophilia due to factor VIII deficiency and hemophilia B/Christmas disease due to factor IX deficiency. Von Willebrand's disease is the most common hereditary disorder characterized by qualitative or quantitative defects in von Willebrand factor. Acquired disorders involve deficiencies of multiple clotting factors and can be caused by vitamin K deficiency, liver disease, or disseminated intravascular coagulation. Coagulation disorders are diagnosed through prolonged coagulation tests and assays showing reduced clotting factor levels. Treatment focuses on replacing the

1. CLOTTING
DISORDER/COAGULATION
DISORDER
Dr. R.RAMYA
1st Year Post Graduate Student
Department of Periodontics and
Implantology

2. DEFINITION
• They are group of disease caused
due to deficiency of clotting factor
and leads to defect in normal clot
formation process.

3. CLASSIFICATION
• Based on origin these disorder are of two types:
HEREDITARY COAGULATION DISORDER:
The inherited plasma coagulation disorders are
due to qualitative or quantitative defect in single
coagulation factor.
ACQUIRED COAGULATION DISORDER:
They are characterised by deficiency of multiple
coagulation factor.

4. HEREDITARY DISORDER
• Sex linked disorder
Eg : classical haemophilia or
haemophilia A
Christmas disease or haemophilia B
• Autosomal Coagulation Disorder:
Eg : Von Willebrand's disease

5. ACQUIRED COAGULATION
DISORDER
• Vitamin K deficiency
• Coagulation disorder of liver disease
• Fibrinolytic defects
• Disseminated intravascular resistance

6. HEREDITARY COAGULATION DISORDER

7. CLASSICAL HEMOPHILIA
• It is the second most common cause of
hereditary coagulation disorder
• It is inherited as x linked recessive trait.so
more common in males while females are
carriers.

8. PATHOGENESIS
• 1. quantitative reduction in factor VIII(In 90%
of cases)
• 2. normal or increased levels of factor VIII(in
10% of cases)
• Factor VIII is synthesized in hepatocytes in
the intrinsic coagulation pathway, factor IXa
complexes with VIIIa, this complex in
presence of platelets, PL and ca2+ activates
factor X to Xa

10. CLINICAL FEATURES
• Symptoms are elicited when factor VIII
reduced to less than 25%.pt suffers bleeding
from hours to days and severity is based on
plasma level of factor VIII
• Recurrent painful hemarthroses and muscle
hematoma and sometimes hematuria

11. LAB DIAGNOSIS
• whole blood coagulation is raised only in
severe cases
• Prothrombin time is usually normal
• Partial thromboplastin time (PTT) is usually
prolonged
• Specific assays for factor VIII shows
reduced activity

12. TREATMENT
• Symptomatic patients with bleeding
episodes are treated with factor 8
replacement therapy consists of factor 8
concentrates or plasma cryoprecipitates.

13. CHRISTMAS
DISEASE/HEMOPHILIA B
• X linked recessive disease
• Rarer than hemophilia A
• Incidence ratio 1:100000 male birth
• inheritance pattern and clinical features are
similar to classical hemophilia

14. LAB DIAGNOSIS &
TREATMENT
• Assay of factor IX is lowered and other lab
findings are similar to hemophilia A
• Infusion of fresh frozen plasma or plasma
enriched with factor ix

15. VON WILLIBRAND’S DISEASE
Most common hereditary coagulation disorder
Inherited as autosomal dominant trait
Incidence ratio 1:1000 of either sex
Occurs due to qualitative or quantitative defect in
VWF
FACTOR VIII- Von Willebrand complex
consist of 99% Vwf of 1% factor VIII , they circulate
together as unit and perform important function in
clotting and facilitate platelet adhesion to
subendothelial collagen.

16. CLINICAL FEATURES
• Spontaneous bleeding from mucous
membrane
• excessive bleeding from nose and
menorrhagia

17. TYPES
• Type1 : it is the most common and
characterized by mild to moderate decrease
in plasma Vwd factor. synthesis is normal
but its release of its multimeter is inhibited
• TYPE 2 : It is much less common and
characterized by normal or near levels of
Vwd factor which was functionally defective
• TYPE 3: It is extremely rare and severe form
of disease. These patients have no
detectable Vwd activity and may have
sufficiently low quantity of factor VIII

18. LAB DIAGNOSIS
• Prolonged bleeding time
• Normal platelet count
• Reduced plasma Vwd concentration
• Defective platelet aggregation with
rostocetin, an antibiotic
• Reduced factor VIII activity
• TREATMENT
• Cryoprecipitates or factor VIII concentrates

19. ACQUIRED COAGULATION DISORDER

20. VITAMIN K DEFICIENCY
• Vitamin k serves as a cofactor in formation of
6 prothrombin complex proteins(vitamin k
dependent coagulation factor) synthesized in
liver. factor II VII IX and x, protein C and
protein S
• CAUSES OF VIT K DEFICIENCY
• Obstructive jaundice
• Chronic diarrhea
• Liver disease
• Hemorrhagic states in infants

21. LAB DIGNOSIS
• Prolonged PTT and APTT
• TREATMENT
• prolonged administration of vitamin k causes
complete recovery in 48 hours

22. COAGULATION DISORDER IN
LIVER DISEASE
• Liver is the site of synthesis and metabolism
of coagulation factors.
• Liver disease leads to hypercoagulability
and predispose DIC and systemic
fibrinolysis.

23. • Major causes of bleeding in liver disease are
• Morphological lesions
• portal hypertension , gastritis and peptic
ulceration
• Hepatic dysfunction
• impaired hepatic synthesis of coagulation
factors and inhibitors , Impaired metabolism
and absorption of vit k and failure to clear
activated coagulation factor
• Complication of therapy
• Massive transfusion leading to platelet and
clotting factor dilution , following heparin
therapy.

24. LAB DIAGNOSIS
• Prolonged PTT and APTT
• Mild thrombocytopenia
• Normal thrombocytopenia and decreased
hepatic stores of vitamin k

25. DISSEMINATED INTRAVASCULAR
COAGULATION
• Also called defibrinate syndrome is a
complex thrombo-hemorrhagic disease
occurring as secondary complication of
some systemic disease.

26. • MAJOR DISORDERS ASSOCIATED WITH
DIC
• Obstetrics complications: abruption
placentae, retained dead fetus, amniotic
fluid embolism.
• Infections: gram negative sepsis
,meningococcemia, rocky mounted spotted
fever and malaria
• Neoplasms: carcinoma of prostate,
pancreas, lung and stomach
• Massive tissue injury: trauma ,burns and
extensive surgery
• Miscellaneous: acute intravascular
hemolysis, snake bite, giant hemangioma ,
shock, heat stroke.

27. PATHOGENESIS
• Although in each case, a distinct triggering
mechanism has been identified, the
sequence of events, in general, can be
summarised as under,
1.Activation of coagulation
2. Thrombotic phase
3. consumption phase
4. Secondary fibrinolysis

28. 1.ACTIVATION OF COAGULATION:
The etiologic factors listed above initiated widespread activation
of coagulation pathway by release of tissue factor.
2. THROMBOTIC PHASE:
Endothelial damage from the various thrombogenic stimuli
causes generalised platelet aggregation and adhesion with resultant
deposition of small thrombi and emboli throughout the microvasculature.
.3.CONSUMPTION PHASE:
The early thrombotic phase is followed by a phase of
consumption of coagulation factors and platelets.
4. SECONDARY FIBRINOLYSIS:
As a protective mechanism, fibrinolysis system is secondarily
activated at the site of intravascular coagulation. Secondary fibrinolysis
causes breakdown of fibrin resulting in formation of FDPs in the
circulation.

30. CLINICAL FEATURES
• Widespread fibrin deposition within
microcirculation leading to ischemia of
organs like brain and kidney.
• BLEEDING DIATHESIS: ensues as platelets
and clotting factors are consumed and there
are secondary release of plasminogen
activator but also digest factor v and VIII
thereby reducing their concentration further.

31. LAB DIAGNOSIS
• Reduced platelet count
• Blood film shows micro-angiopathic
hemorrhagic hemolytic anemia
• PTT,APTT and TT are prolonged
• Plasma fibrinogen level is reduced
• TREATMENT
• Anticoagulants like heparin or coagulants
contained in fresh frozen plasma
• Underlying disorders must be treated
simultaneously

32. THANK YOU