Bleeding disorders are medical conditions that cause abnormal or excessive bleeding due to defects in the blood clotting process. The document discusses the main types of bleeding disorders: platelet disorders like thrombocytopenia and thrombocytosis; vessel wall abnormalities like scurvy and hereditary hemorrhagic telangiectasia; and clotting disorders like hemophilia A, hemophilia B, and von Willebrand disease. Specific conditions are defined and their signs, symptoms, causes, diagnosis, treatment and dental implications are explained. The document provides an overview of different bleeding disorders, their etiology and clinical manifestations.

1. Presented by :
Wafa Ahmed Ehmoda
Najla Mohammed Sasi
Bleeding disorders

2. Bleeding Disorder
 Def : Bleeding disorders is a general term for
a wide range of medical problems that lead to
poor blood clotting and continuous bleeding.
 characterized clinically by abnormal bleeding,
which can either be spontaneous or become
evident after some inciting event.
 can result from :
 defects in the blood vessel
 abnormalities in the blood itself
 blood clotting factor
 platelets
Source from : National Haemophilia Association

3. Etiology
I) PLATLETS DISORDER
II) VESSEL WALL ABNORMALITIES
III)CLOTTING DISORDER

4. i) PLATELET DISORDER, what
is it?

5. WHAT IS PLATELETS ?
 Oblong disk shape
 Size- 2-4 µm
 Produced in bone marrow by megakaryocte cell
 Platelet count in blood- 150,000-350,000 µL
 Life span- mean survival time (8_10) days
 Function :-
 a- Aggregation & adherence to injured vessel to form
primary clot (plug)
 b- help in clot formation

6. Platelets Disorders
Thrombocytopenia Thrombocytosis Platelet dysfunction

7. THROMBOCYTOPENIA
 Defined as reduced in the platelet count< 150, 000µL that
characterized by spontaneous bleeding, a prolonged bleeding
time, and a normal PT and PTT.
 The risk of bleeding depends on the level of the platelet count:
Mild
thrombocytopenia
(platelet <150 000
cells/µL)
Moderate
thrombocytopenia
(platelet 20 000 - 50
000 cells/µL)
Severe
thrombocytopenia
(platelet <20 000
cells/µL)

8. Etiology:
I.Idiopathic (ITP):
a.Increased platelets destruction.
b.Decreased platelets production.
c.Both.
II.Secondary to:
a.Drugs induced thrombocytopenia→ e.g (cytotoxic drugs)
b.Secondary to some diseases:
• Bacterial or viral infection.
• Uremia,SLE.
• Neoplasia of bone marrow or lymphoid tissues
(leukemia,lymphomas).
• Radiotherapy .

9. CLINICAL MANIFESTATIONS
 Onset is usually sudden for acute ITP and in chronic ITP, it is
insidious onset.
 Petechiae or purpura
 Feet, legs, arms, and buttocks.
 Mucosal bleeding.
 Palatal petechiae, epistaxis, hematuria, menorrhagia, GI
bleeding.
 Gingival bleeding

11. DIAGNOSIS
• History taking.
• Physical examination.
o Signs of bleeding (petechiae and purpura).
o Mucosal bleeding.
• Investigations.
o Full blood count.
Low platelet count.
o Histological findings.
Platelets are normal in size or may appear
larger than normal.
Normal red blood cells morphology.
Normal white blood cells morphology.
o Coagulation tests.

12. TREATMENT &
MANAGEMENT
1.Remove the underlying cause if known.
2.Corticosteroids are useful in iodiopathic
thrombocytopenia purpura (ITP) and in autoimmune
drugs induced thrombocytopenia.
3.Splenectomy.
4.Platelets transfusion for secondary thrmbocytopenia.
5.Neumega is a new drug used to stimulate platelets in
bone marrow.

13. Dental implication:
 Gingival bleeding can be controlled by the use of fibrin
foam,gel foam or absorbable cellulose with thrombin.
 20 vol.hydrogen peroxide should be tried , in many
cases it will control gingival oozing.
 Elective dental surgery should be deferred.

14. Thrombocytosis
thrombocythemia
There is increased number of circulating platelets , the
elevation is in the range of 500,000_1000,000/cubic
mm, but may be higher.
Etiology:
1.idiopathic.
2.secondary: may be secondary to ;
 Splenectomy.
 Myeloproliferative disease

15. Dental implication:
1.Dental treatment should be conservative, elective traetment
should be delayed.
2.If there is gingival bleeding associated with gingivitis or
periodontitis ,only scaling is performed to one quadrant per
visit with extreme care to avoid inflicting trauma to the
gingival tissue.
3.Hydrogen peroxide (20 vol.)and local hemostatic agents
should be tried to stop gingival bleeding.
4.If extraction is unavoidable , pack the socket with gel-foam
which is usually sufficient to stop the bleeding.
5.The dental management is complicated by hemorrhage,
thrombosis and cytotoxic agents.

16. Disorder Of Platelets
Function
•Congenital platelets disorders ↓↓↓
•Defect in adherence :-
>Bernard-Soulier syndrome
• The glycoprotein (Ib) receptor for VIII:Vwf is
absent on platelet membrane.
• Rx: platelets transfusion.
•Defect in aggregation :-
>Glanzmann thrombasthenia
the platelets lack surface glycoprotein
receptor(IIb,IIIa)necessary for binding
fibrinogen(fibrinogen receptor)
• Bleeding time is prolonged

17. Disorder of platelets function
•Acquired platelets disorders ↓↓↓
1..Meloproliferative diseases
2.. UREMIA
3.. Dysproteinemia
4..Drugs (Aspirin _Clopidogrol)

18. II) Vessel WALL abnormalities
 SCURVY
 INFECTIONS (measles, scarlet fever,
endocarditis,malaria)
 ALLERGY
 HEREDITARY HEMORRHAGIC TELANGIECTASIS

19. Heridetary hemorrhagic
telangectsis
 Dominant inherited condition , there is a telengectiasis
and small aneurysms found on finger tips, face,nasal
passages, tongue and GIT.
 Small group of people develope pulmonary A/V
malformation.
 Pt either develops reccurent bleeding /epistaxis/iron def.
anemia due to GIT bleeding.
 Rx ↓
 Iron therapy for blood loss.
 Local cautery/ laser therapy for single lesion from
bleeding (epistaxis).

20. Dental aspect:
 Trauma should be avoided whenever possible.
 Lesions may occasionally interfere with periodontal
therapy or oral surgery and the bleeding can be treated
by electrocoagulation, laser therapy, cryotherapy or
resection.
 Escharotic agents such as silver nitrate, 50%
trichloroacetic acid and chromic acid are effective on
only small bleeding points.
 Sclerosing agents such as prophylaxis.
 Iron and folate therapy may be required if there is
chronic anemia.
 Non steroidal anti-inflammatory analgesics are
contraindicated because of the risk of gastrointestinal

21. Vessel wall abnormalities
Ehlers danlos disease :
 Congenital disorder of collagen synthesis in which capillaries
are poorly supported by collagen and ecchymosis are
commonly observed.

22. III) Clotting disorder
• Haemophilia
• von Willerbrand
Disorder
Primary
(Inherited
)
• Vitamin K
Deficiency
• Hepatic Failure
• DIC
Secondar
y
(Acquired
)

23. Haemophilia – overview
 A group of blood disorders in which there is defect in clotting
factors.
 70% are X-linked recessive disorder. 30% spontaneous
mutation.
 The bleeding patterns of haemophilia are similar.
 Types :
 A:Deficiency in factor VIII (classic haemophilia).
 B: Deficiency in factor IX (Christmas disease).
 C: Deficiency in factor XI.

24. Hemophilia A (classic or true
hemophilia)
Definition:
This is most common type of hemophilia ,it is
characterized by deficiency of factor VIII and prolonged
(APTT).
Normal value of VIII is 50% _150%.
Normal value of (APTT) is 25 _40 sec.
Bleeding character in hemophilia:
bleeding stop immediately after injury as a result of normal
vascular and platelets response but after an hour or
more intractable oozing or rapid blood loss starts and
persists.

25. Haemophilia - classification
Classification Clinical Manifestation
Severe
(<1% of normal)
• Manifest in infancy when child reaches toddler
stage
• Spontaneous bleeding – in muscles or joints
(haemarthroses)
• Excessive bleeding after minortrauma,
postoperatively, or after intramuscular childhood
vaccinations.
Moderate
(1-5% of normal)
• Manifest after2 years of life
• Moderate trauma causes bleeding episodes
• Occasionally spontaneous bleeding occurs
Mild
(>5% - <40% of
normal)
• Often diagnosed in teenagers and adults
• Significant trauma to induce bleeding
• No spontaneous bleeding

26. 26
Haemophilia – clinical
manifestation
 Haemarthrosis (spontaneous bleeding in
muscle or joints - painful)
 Joint Swelling
 Easy bruising
 Epistaxis
 Haematuria
 Intracranial hemorrhage

27. Oral manifestations
 1- bleeding from any site of oral cavity and sometimes it may
cause respiratory obstruction.
 2- gingival hemorrhage.
 3- bleeding associated with physiological erupation.
 4- tooth extraction or minor surgery may lead to sever
hemorrhage, sometimes may be fatal.

28. Dental management of
hemophilia A
 Prophylactic measures:-
 1-regular dental care.
 2- fluoride application.
 3- sugar restriction.
 4-prevention of periodontal disease.
 These measures reduce the need for extraction which may be
major hazard in hemophilia.
 Endodontics: reaming through the apex should be avoided.
 Periodontal therapy: scaling can be performed(except in sever
hemophilia)under antifibrinolytic cover.
 Conservative dentistry: care should be considered to avoid soft
tissue trauma.

29. HAZARD OF ANASTHESIA :
 Local and general anesthesia are hazardous in absence of factor
replacement .
 Anesthesia injection especially nerve block can be hazard
particularly for severe hemophiliacs, bec the needle tear the wall of
small blood vessels and a deep spreading hematoma can threaten
the air way also the hematoma is formed during intramuscular
injection so the best safer is nitrous oxide analgesia to avoid use of
L.A.
 MANAGEMENT OF EXTRACTION:
 1-Laboratory tests : factor VIII should be raised to 50%_70%before
dental extraction and raised to 100% for major surgery.
 2-postoperatively the patient should be hospitalised and factor VIII
should be given I.V.

30. Hemophilia B CHRISTMAS
DISEASE
The disease is identical to hemophilia A but charaterised by
deficiency of factor IX.
TREATMENT :-
1- mild hemophilia B :- fresh frozen plasma is adequate.
2-severe hemophilia B:-factor IX (I.V) 1hr preoperatively.

31. Von willebrand‘s disorder -
overview
 Most common hereditary deficiency caused abnormality
in von Willerbrand protein.
 Functions on both primary & secondary homestasis.
 1. To act as bridge between subendothelial collagen
and platelets
 2. Bind and protect factorVIII from rapid clearance
then delivers it to site of injury.

32. Von willebrand‘s disorder -
types
 Type 1 _ mild reduction of factor VIII:vWF
 Type 2 –molecular defect of VIII:vWF
 Type 3 – nearly no detectable level of factor VIII:vWF and
therefore factor VIII:C.

34. Clinical menifestation
 Asymptomatic .
 Mucous membrane bleeding.
 Epistaxis
 Cutaneus bleeding.
 Gingival bleeding
 Menorrhagia

35. investigation
 Full Blood Count – platelet normal
 APTT PROLONGE or normal
 Factor VIII LOWor normal.
 von Willerbrand Factor activity (ristocetin
cofactor)
 Ristocetin, an antibiotic that causes vWF to bind to
platelet taken from plasma.
 In healthy people, platelet rapidly agglutinate.
 von Willerbrand Factor antigen
 Measure vWF protein and binding sites.
 Not accurate.

36. treatment
1-Mild-Moderate vWD:
 Desmopressin forminorsurgery.
 Fresh frozen plasma.
 Cryoprecipitate.
2- in severe vWD: factorVIII concentrate formajorsurgery.

37. Prognosis & complications
 Lifelong tendency toward easy bruising, frequent epistaxis,
and menorrhagia.
 Carry medic-alert bracelet or chain & carry books diagnosis,
types etc.

39. Hepatic faliure
In addition to vit k dependent factors(II, VII, IX ,X) the liver
synthesizes fibrinogen ,plasminogen,factors
(V,VIII,XI,XIII).
Liver disease may be associated with increased bleeding
tendency and this may be due to:
1-reduced vit K absorption.
2-reduced synthesis of coagulation factors.
3-increased fibrinolysis.
4-thrombocytopenia.
5-viral hepatitis or alcoholism .
6-DIC.

40. Management
 In mild liver disease : vit K may be effective.
 In sever cases : tranxemic acid and fresh plasma may control
bleeding.
 REYE‘S SYNDROME :
 There is some evidence that use of aspirin in children up to the ag
of
15 yrs ,may develop rarely liver damage(diffuse microvascular fatty
infiltration)and acute encephalopathy with cerebral edema.

41. Vitamin k deficiency
 Required for synthesis of Plasma factors II, VII, IX, and X
Causes of vit k deficiency:
1-poor dietary intake.
2-poor absorption.
3-failure of utilization.
4-lack of vit k synthesis ;prolong use of broad spectrum
antibiotics.
General manifestation :
1-Bruising 2-GIT bleeding 3-hematouria 4-cerebral
bleeding
Management :

42. ANTICOAGULANTS
Anticoagulants are drugs used for prevention and treatment of
thrombosis e.g : DVT ,myocardial infarction ,renal dialysis
and cerebral thrombosis.
Anticoagulant drugs includes :
1-oral anticoagulant.
2-heparin.
Oral anticoagulants ; cumarin “warfarin”
Action : Inhibition of the enzyme “vit K epoxide reductase”
Cumarin leads to the inhibition of synthesis of biologically active
prothrombin factor II and factors VII,IX and X.

43.  LABORATORY FINDINGS :
1-prolonged APTT
2-Prolonged PT (normal value is 11_15 sec)
Management :-
 In minor oral surgery the PT should be within the normal
therapeutic range (1.5-2.5 times the normal).
 INR should be checked on the day of operation or a day
before.
 The therapeutic range of INR is 2-3.5.
 The surgery should be a traumatic as possible.
 If any sign of bleeding from extraction socket ,use oxidized
cellelouse,with suture over the socket (eg. Figure 8 suture)and
pressure pack is applied.

44. Heparin
Action:
prevent fibrin formation through:
a. Inhibition of thrombin-fibrinogen reaction.
b. Inactivate factors 1Xa,Xa,X1a, and X11a.
c. Prevention of platelets aggregation (large dose).
Management :
1.Surgery can be safely carried out ,when the effect of
heparin has ceased.
2.In renal dialysis, surgery is better carried out the next
day.

45. Disorder characterized by coagulation pathway
activation leading to diffuse fibrin deposition in the
microvasculature and consumption of coagulation
factors and platelets.
 Occurs as secondary complication of variety
diseases.
 Caused by the systemic activation of coagulation
pathways, leading to formation of thrombi
throughout the microcirculation and widespread
thromboses. There is consumption of platelets and
coagulation factors and secondarily activation of
Disseminated IntravascularCoagulation (DIC)

46. Pathophysiology of DIC
Massive tissue
destruction
sepsis
Endothelial
injury
Release of tissue
factor Platelet
aggregatio
n
Widespread
microvascular thrombosis
Consumption
of clotting
factors and
platelets
Ischemic tissue
damage
fibrinolysis
Vascular
occlusion
Microangiopathi
c hemolytic
anemia
Activation of
plasmin
Proteolysis
of clotting
factor
Fibrin split
products
Inhibition of thrombin, platelet
aggregation and fibrin
polymerization

47. DIC
Precipitating factors:
Including incompatible blood transfusion, severe
sepsis,severe trauma or burns and cancer (metastatic
cancer of the pancreas, lung, stomach or prostate).
The possible effect of DIC includes:
a.Hemorrhagic tendencies.
b.Thrombotic phenomenon.
c.Hemolysis of red cells.
d.Shock.

48. Clinical features
Bleeding, thrombosis,bleeding far from common than
thrombosis.
Subacute DIC :
Occures primarily in cancerous pts results in superficial +deep
venous thrombosis.
Other manifestation :
High incidence of cardio respiratory faliure.

49. Treatment
 Treat the disorder inducing the DIC first such as sepsis.
 Support the patient by correcting hypoxia, acidosis and poor
perfusion.
 Replace depleted blood clotting factors, platelets and
anticoagulant proteins by transfusion.
 Heparin may be used to treat significant arterial or venous
thrombotic disease unless sites of life-threatening bleeding
coexist. Thus, the use of heparin remains controversial.
 Treatment with anticoagulants or coagulants contained in
fresh-frozen plasma usually needed in acute case.
 Drotregocin alfa (recombinant activated protein C) reduces
mortality in adults with DIC and sepsis.

50. Diagnosis of patient with
bleeding disorders
An adequate history is the single most important part of the
evaluation of Pts with abnormal bleeding tendency, clinical
examination is also necessary, but the hematological tests are
needed to confirm the diagnosis.

51. Diagnosis of patientswith
bleeding disorders
History
1.Bleeding problem in relatives.
2.Bleeding problem following trauma.
3. Bleeding problem following operation.
4. Medication that may cause bleeding problems.
5.Presence of disease that may have associated bleeding
problems.

52. Diagnosis of patients with
bleeding disorders
Clinical Examination
oThe skin and mucous membranes should be examined for
:
petechia, ecchymosis, hematoma, angiomas and jaundice.
oThe lymph nodes should be examined and mobility of the
joint should be observed.

53. Diagnosis of patients with
bleeding disorders
Screeing Laboratory TESTS
Hemgram
PT
BT
PTT
TT

54. 2) Screening test
Test Mechanism Tested Normal Value Disorder
Bleeding time
(BT)
Hemostasis,
capillary & platelet
function
3-7 min beyond
neonate
Thrombocytopenia
, von Willebrand
disease
Platelet count Platelet number 150 000 – 450
000 / mm^3
Thrombocytopenia
Prothrombin
time
(PT)
Extrinsic & common
pathway
< 12 sec beyond
neonate; 12-18 sec
in term neonate
Defect in Vit K-
dependent factor,
liver disease, DIC
Activated
partial
thromboplastin
time
(APTT)
Intrinsic & common
pathway
25-40 sec beyond
neonate; 70 sec in
term neonate
Hemophilia, von
Willebrand
disease, DIC
Source from : Nelson Essential of Pediatrics 5th
edition

55. Thank
you