The document discusses platelet disorders, including thrombocytopenia and thrombocytosis, outlining their definitions, etiologies, clinical manifestations, and diagnostic approaches. It details the management and treatment options for these disorders, as well as implications for dental care, particularly in hemophilia and von Willebrand's disease. The text emphasizes the importance of a thorough history and clinical examination in diagnosing bleeding disorders.

1. BY Dr. ARUNA
SENIOR RESIDENT
PATHOLOGY
KIMS
PLATELET DISORDERS

2. INTRODUCTION:
 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 :
1. defects in the blood vessel
2. abnormalities in the blood itself- clotting factors and platelets

3. Etiology
 PLATLETS DISORDER
 VESSEL WALL ABNORMALITIES
 CLOTTING DISORDER

4. i) PLATELET DISORDER, what is
it?

5. WHAT IS PLATELET ?
 Oblong discshape
 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 :- Aggregation & adherence to injured vessel
to form primary clot (plug)
 help in clot formation

6. PLATELETS DISORDERS
THROMBOCYTOSIS THROMBOCYOTOPENIA
PLATELET
DISORDERS

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.

8. Mild
thrombocytopenia
• platelet <150 000 cells/µL
Moderate
thrombocytopenia
• platelet 20 000 - 50 000 cells/µL
Severe
thrombocytopenia
• platelet <20 000 cells/µL
The risk of bleeding depends on the level of the
platelet count

9. Etiology:
Idiopathic (ITP):
1. Increased platelet destruction
2. Decreased platelets production.
3. Both.
Secondary to:
1. Drugs induced thrombocytopenia→ e.g (cytotoxic drugs)
 Secondary to some diseases
1. Bacterial or viral infection.
2. Uremia, SLE.
3. Neoplasia of bone marrow or lymphoid tissues (leukemia, lymphomas).
4. Radiotherapy .

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

11. Purpura on legs

12. Petechiae on legs

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

14. TREATMENT & MANA GEMENT
1. Remove the underlying cause if known.
2. Corticosteroids are useful in idiopathic,
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 inbone marrow.

15. 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.

16. 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:
 idiopathic
 secondary: may be secondary to
1. Splenectomy.
2. Myeloproliferative diseases

17. 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

18. Disorder Of Platelets Function
 Acquired platelets disorders ↓↓↓
1. Meloproliferative diseases
2. UREMIA
3. Dysproteinemia
4. Drugs (Aspirin .Clopidogrol)

19. Vessel Wall Abnormalities
1. SCURVY
2. INFECTIONS (measles, scarlet fever,
endocarditis,malaria)
3. ALLERGY
4. HEREDITARY HEMORRHAGIC
TELANGIECTASIS

20. 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 ↓
1. Iron therapy for blood loss.

21. 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, cryo-therapy or resection.
Escharotic agents such as silver nitrate, 50% trichloro-acetic 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

22. Vessel Wall Abnormalities
EHLERS DANLOS DISEASE :
Congenital disorder of collagen synthesis in which capillaries
are poorly supported by collagen and ecchymosis are
commonly observed.

23. Clotting Disorder
• HAEMOPHILIA
• vWD
PRIMARY-
INHERITED
• HEPATIC FAILURE
• VIT-K DEF
• DIC
SECONDARY-
ACQUIRED

24. Haemophilia – overview
1. A group of blood disorders in which there is defect in clotting
factors.
2. 70% are X-linked recessive disorder. 30% spontaneous
mutation.
3. The bleeding patterns of haemophilia are similar.
Types :
 A:Deficiency in factor VIII (classic

25. 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

26. SEVERE
• Manifest in infancy when child
reaches toddler stage
• Spontaneous bleeding – in muscles
or joints (haemarthroses)
• Excessive bleeding after minor
trauma,
• postoperatively, or after
intramuscular childhood
vaccinations.
MODERATE
• Manifest after 2
years of life
• Moderate trauma
causes bleeding
episodes
• Occasional y
spontaneous
bleeding occurs
MILD
• Often diagnosed in
teenagers and
adults
• Significant trauma
to induce bleeding
• No spontaneous
bleeding
HAEMOPHILIA-CLASSIFICATION

27. 26
Haemophilia – Clinical Manifestation
 Haemarthrosis (spontaneous bleeding
in muscle or joints - painful)
 Joint Swelling
 Easy bruising
 Epistaxis
 Haematuria
 Intracranial hemorrhage

28. 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.

29. Dental management of hemophilia A
Prophylactic measures:-
1. regular dental care.
2. fluoride application.
3. sugar restriction.
4. prevention of
periodontal disease.
5. These measures reduce the need for extraction which may be major
hazard in hemophilia.
6. Endodontics: reaming through the apex should be avoided.
7. Periodontal therapy: scaling can be performed(except in sever
hemophilia)under antifibrinolytic cover.

30. 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:
1Laboratory tests : factor VIII should be raised to 50%_70%before dental
extraction and raised to 100% for major surgery.
□
□
□
□

31. 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.

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

33. 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.
□
□

35. Clinical manifestation
□ Asymptomatic .
□ Mucous membrane bleeding.
 Epistaxis
□ Cutaneus bleeding.
 Gingival bleeding
□ Menorrhagia

36. investigation
□ Complete Blood Count – platelet
normal
□ APTT PROLONGED 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

37. treatment
1-Mild-Moderate vWD
:
❖
❖
❖ Desmopressin for minor
surgery. Fresh frozen plasma.
Cryoprecipitate.
2- in severe vWD : factor VIII concentrate for major
surgery.

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

40. 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.
2reduced synthesis of coagulation
factors. 3-increased fibrinolysis.
4 thrombocytopenia.
5iral hepatitis or alcoholism .

41. 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 a of
15 yrs ,may develop rarely liver damage(diffuse microvascular
fatty infiltration)and acute encephalopathy with cerebral
edema.

42. 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

43. 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
Disseminated Intravascular Coagulation
(DIC)

44. Pathophysiology of DIC
Massive
tissue
destruction
sepsis Endotheli
al
injury
Release of tissue
factor Platelet
aggregati
o n
Widespread
microvascular
thrombosis
Consumptio
nof clotting
factors
and
platelets
Ischemic
tissue
damage
fibrinolysis
Vascular
occlusio
n
Microangiopat
hi c hemolytic
anemia
Activation
ofplasmi
n
Proteolysi
s of
clotting
factor
Fibrin
split
products
Inhibition of thrombin,
platelet aggregation and
fibrin polymerization

45. 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.

46. 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.

47. 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
❑
❑
❑
❑
❑

48. 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.

49. 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

50. 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.

51. Diagnosis of patients with
bleeding disorders
□Screeing Laboratory TESTS
❖Hemgram
❖PT
❖BT
❖aPTT
❖TT

52. Thank
you