HEMORRHAGIC

DISORDERS IN
CHILDREN….

PRESENTED BY DR. ASHWIDA RAMLAN

GUIDED BY DR. SOWMYA SHETTY

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 Contents
•INTRODUCTION
•CLASSIFICATIONS OF BLEEDING DISORDERS
•EVALUATION OF THE PATIENT WITH HEMORRHAGIC
DISORDERS
•LABORATORY TESTS, THEIR NORMAL VALUES AND
COMMON CAUSES OF ABNORMALITIES
•TESTS OF VASCULAR AND PLATELET PHASES
•LABORATORY FINDINGS IN VARIOUS PLATELET AND
COAGULATION DISORDERS
•BLEEDING DISORDERS
-COAGULATION FACTOR DEFICIENCIES
-PLATELET DISORDERS
-VASCULAR DISORDERS
-FIBRINOLYTIC DISORDERS
•HOME REMEDIES TO CONTROL BLEEDING
•CONCLUSION
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•REFERENCES
 INTRODUCTION
•Blood is a body fluid that delivers necessary substances such
as nutrients and oxygen to the cells and transports metabolic
waste products away from those cells.

•FUNCTIONS OF BLOOD
1.TRANSPORTATION
2.PROTECTION
3.REGULATION

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Transportation
•Blood is the primary means of transport in the body that is
responsible for transporting important nutrients and materials to and
from the cells and molecules that make up our body.
•Transports oxygen processed by the lungs to all the cells of the body
and then to collect the carbon dioxide from the cells and deliver it to
the lungs.
• collects metabolic waste from up and down the body and take it to
the kidneys for excretion.
•Delivers the nutrients and glucose generated by the organs of the
digestive system to the other parts of the body including the liver.
•Transportation of hormones produced by the glands of the
endocrine system.

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  Protection
•Protects the body from infections.
• The white blood cells found in blood are responsible for
safeguarding the different organs of the body by
producing antibodies and proteins which are capable of
fighting off and killing the germs and viruses that can
causes serious damage to the body cells.
•The platelets limits blood loss by helping the blood to
clot quickly.

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Regulation
•Blood is a regulator of many factors in the body.
Maintains the temperature to a level that is tolerated by
the body with ease.
• Blood is responsible for controlling the pH balance.
•Another regulatory task performed by blood is to control
the blood pressure and restrict it under a normal range.

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EFFECTS OF loss of blood
The effect of blood loss depend upon 3 main factors:
The amount of blood loss
The speed of blood loss
The site of blood loss.

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 Cardiovascular Respiratory Neurologic Skin renal
Minimal Blood Slightly Warm and pink
Loss. anxious
Blood
Volume Loss
<15%

 Mild Blood Tachycardia, Mild  Irritable Cool Oliguria

Loss. Diminished peripheral Tachypnea Confused extremities,
Blood pulses Mottling,
Volume Loss: Delayed 
15-30%
capilary Refill

Moderate Significant   Tachypnea Irritable, Cool Oliguria

Blood Loss. Tachycardia, Lethargic, extremities,
Blood Volume Thready peripheral Diminished mottling or
Loss: 30-40% pulses,
Hypotension pain pallor
Metabolic Acidosis response

Severe Blood Severe Tachycardia tacyhpnoea Lethargic Cold Anuria

Loss Thready central pulses, Coma extremities
Blood Significant  Pallor
Volume Loss:
Hypotension, Cyanosis
>40% Significant acidosis

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 HEMORRHAGE
•Hemorrhage is the escape of blood from a blood vessel.
•Extravasation of blood into the tissues with resultant
swelling is known as hematoma.
•Large extravasation of blood into skin and mucus
membrane are called echymoses.
•Purpuras are small areas of hemorrhages (upto 1 cm)
into the skin and mucus membrane.
•Petechiae are minute pinhead sized hemorrhages.

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 HEMORRHAGIC DISORDERS
•The hemorrhagic disorders are a group of disorders of widely
differing etiology, which have in common an abnormal tendency to
bleed due to a defect in the mechanism of hemostasis.
•A number of dental procedures result in the
risk of bleeding that can have serious consequences, such as
severe hemorrhage, or possibly death, for the patient with a
bleeding disorder.
•Safe dental care may require consultation with the patient’s
physician, systemic management and dental treatment
modifications.

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 CLASSIFICATION OF HEMORRHAGIC
DISORDERS
1. BASED ON THE TYPE OF BLOOD VESSEL INVOLVED:
a. Arterial hemorrhage: there is bleeding from a ruptured artery.
Arterial bleeding is pulsatile, brisk and bright red in colour.
b. Venous hemorrhage: loss of blood from a vein. Bleeding from
veins is dark in colour and blood flows in an even stream.
c. Capillary hemorrhage: oozing of blood from capillaries. Blood is
bluish red in colour . Bleeding is not severe and is easily
controlled by simple pressure with gauze pads.

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2. BASED ON THE TIME OF BLEEDING:
a. Primary bleeding: occurs at the time of injury
b. Secondary bleeding: if the primary bleeding has stopped once
and wound starts to bleed again after 24 hours to several days.
c. Intermediate bleeding: bleeding occurring within 8 hours after
stoppage of primary bleeding.

3. INTERNAL OR EXTERNAL BLEEDING:

d. Internal bleeding: bleeding that is confined within the body
cavity and is not apparent on the surface.
e. External bleeding: blood escaping through a wound in the skin.

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4. BASED ON THE DEFECT IN NORMAL HEMOSTASIS
 Vessel wall disorders
• Scurvy
• Purpura
• Hereditary hemorrhagic telangiectasia/ Ehlers-Danlos syndrome
 Platelet disorders
• Congenital
o Thrombocytopenic – quantitative platelet deficiency
May- hegglin anomaly
Wiskott- Aldrich syndrome
Neonatal alloimmune thrombocytopenia
o Nonthrombocytopenic- qualitative or functional platelet defect
Glanzmann’s thrombasthenia
von Willebrand’s disease
Bernard- Soulier syndrome

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•Acquired
oThrombocytopenic – quantitative platelet deficiency
autoimmune or idiopathic Thrombocytopenic purpura
Thrombotic Thrombocytopenic purpura
cytotoxic chemotherapy
Drug induced
Leukemia
Aplastic anemia
Myelodysplasia
Systemic lupus erythematosus
Associated with infection: HIV, mononucleosis, malaria
Disseminated intravascular coagulation
o Nonthrombocytopenic- qualitative or functional platelet defect
Drug induced
Uremia
Alcohol dependancy
Liver disease
Myeloma, myeloproliferative disorders, macroglobulinemia
Acquired platelet- type von willebrand’s disease

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 Coagulation disorders
• Hemophilia A and B
• von Willebrand’s disease
• Other factor deficiencies (rare)

 Fibrinolytic disorders
• Streptokinase therapy
• Disseminated intravascular coagulation

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5. BASED ON THE CAUSES OF HEMORRHAGE:
1.Local cause
•Trauma
•Surgeries
2. Systemic cause
•Spontaneous hemorrhage; eg. Rupture of an aneurysm,
septicemia, bleeding disorder
•Inflammatory lesions of the vessel wall. Eg. Bleeding from
chronic peptic ulcer
•Neoplastic invasion
•Vascular diseases such as atherosclerosis
•Elevated pressure within the vessels.

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Traumatic Injury
Traumatic bleeding is caused by some type of injury. There are different types
of wounds which may cause traumatic bleeding. These include:
Abrasion - Also called a graze, this is caused by transverse action of a foreign
object against the skin, and usually does not penetrate below the epidermis
Excoriation - In common with Abrasion, this is caused by mechanical destruction
of the skin, although it usually has an underlying medical cause
Hematoma - Caused by damage to a blood vessel that in turn causes blood to
collect under the skin.
Laceration - Irregular wound caused by blunt impact to soft tissue, overlying hard
tissue or tearing such as in childbirth.
Incision - A cut into a body tissue or organ, such as by a scalpel, made during
surgery.
Puncture Wound - Caused by an object that penetrated the skin and underlying
layers, such as a nail, needle or knife
Contusion - Also known as a bruise, this is a blunt trauma damaging tissue under
the surface of the skin
Crushing Injuries - Caused by a great or extreme amount of force applied over a
period of time. The extent of a crushing injury may not immediately present itself.
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6. BASED ON THE AMOUNT OF BLOOD LOSS

ACUTE BLOOD LOSS:

Sudden loss of blood
May cause hypovolemic shock
May lead to death

CHRONIC BLOOD LOSS:

Loss of blood volume over a period of time.
May not be necessarily fatal
Generally produces iron defeciency anemia

A sudden loss of 33% blood volume may cause death, while loss of
upto 50% blood volume over a period of 24 hours may not be
necessarily fatal.
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 EVALUATION OF PATIENT WITH
HEMORRHAGIC DISORDERS
In order to establish a definite diagnosis in any case
suspected to have abnormal hemostatic functions, the
following scheme is folowed:
1. Comprehensive clinical evaluation, including the
patient’s history, family history and details of the site,
frequency and character of hemostatic defect.
2. Series of screening tests for assessing the
abnormalities in various components involved in
maintaining hemostatic values.
3. Specific tests to pinpoint the cause.
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 SCREENING TESTS FOR CLOTTING
• Bleeding time
• Clotting time
• Platelet count
• Thrombin time
• Activated partial thromboplastin time

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BLEEDING TIME: it is the time taken from puncture of the blood vessel to the
stoppage of bleeding.
Procedure:
1. Duke’s method: convenient and commonly
used.
 Set the stop watch at 0.
 The tip of the finger is cleaned with spirit
and allowed to dry. Make a puncture deep enough
(about 3 to 4 mm) to ensure free flow of blood
without squeezing.
 Immediately start the stop watch and note the time.
 30 sec later escaping blood is dried on the edge of a
clean piece of filter paper.
 Repeat the procedure for every 30 sec until
bleading ceases and no further blood spot appear
on the filter paper.
 Therefore, each blot of blood on the filter paper represents 30 sec flow of blood.
 Normal bleeding time by this method is 2 to 6 minutes

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2. Ivy’s method:
Clean the anterior surface of the
forearm with spirit.
Apply pressure of 40mmHg to
the upper arm with a
sphygmomanometer
and maintain this pressure till the
end of the experiment.
Sterilize the needle and make a puncture deep into the skin on the
anterior surface of the forearm. Remove the drop of blood on filter
paper as in duke’s method.
Normal bleeding time by this method is 2 to 10 minutes.

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CLOTTING TIME: It is the time taken from the puncture of the blood vessel to
the formation of a fibrin thread.
PROCEDURE:
1. CAPILLARY GLASS TUBE METHOD:
 Set the stop watch at 0.
 The tip of the finger is cleaned with spirit and allowed to dry. Make a puncture
deep enough (about 3 to 4 mm) to ensure free flow of blood without squeezing.
 Immediately start the stop watch and note the time.
 When a large drop of blood has collected, introduce the end of the capillary tube
into the drop holding the tube such that its other end will be at a lower level. Blood
flows rapidly into the capillary tube.
 Hold the capillary tube filled with blood in the palm of the hand so as to maintain it
at body temperature.
 At the end of 1 minute, break off about 1 cm
of the tube from 1 end and notice if a thread of fibrin
connects the broken ends of the tube. If there is no fibrin
thread, repeat the procedure every 30 seconds till a fibrin
thread appears. The appearance of the fibrin thread of
about 5 mm length indicates that the blood has clotted.
 Normal value by this method is 3 to 8 minutes.
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PLATELET COUNT: it is a routine blood laboratory test that provides a
quantitative assessment of circulating platelets in the vascular system.

THROMBIN TIME:
The thrombin time (TT), also known as the thrombin clotting time (TCT) is a blood
test that measures the time it takes for a clot to form in the plasma of a blood sample
containing anticoagulant, after an excess of thrombin has been added.
The thrombin time compares the rate of clot formation to that of a sample of normal
pooled plasma. Thrombin is added to the samples of plasma. If the time it takes for the
plasma to clot is prolonged, a quantitative (fibrinogen deficiency) or qualitative
(dysfunctional fibrinogen) defect is present.
Normal values for thrombin time are 24 to 35 seconds.

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PROTHROMBIN TIME:
The prothrombin time is most commonly measured using blood plasma.
Blood is drawn into a test tube containing liquid sodium citrate, which acts as an
anticoagulant by binding the calcium in a sample. The blood is mixed, then centrifuged to
separate blood cells from plasma. 
The plasma is analyzed by a laboratory technician on an automated instrument at 37 °C (as
a nominal approximation of normal human body temperature), which takes a sample of the
plasma. An excess of calcium is added (thereby reversing the effects of citrate), which
enables the blood to clot again.
Tissue factor (also known as factor III) is added, and the time the sample takes to clot is
measured optically. 
Normal : 11 to 15 sec.

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ACTIVATED PARTIAL THROMBOPLASTIN TIME:
This test is used to measure the activity of intrinsic and common pathway
factors.
The test consists of addition of 3 substances to the plasma; calcium,
phospholipid and a surface activator such as kaolin.
Addition of calcium initiates clotting and timing begins. The APTT is the time
taken from the addition of calcium to the formation of a fibrin clot.
The normal range is 25 to 40 secs.

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 NORMAL VALUES
TEST NORMAL RANGE

Platelet count 1,50,000 to 4,50,000/cu mm

Ivy Bleeding time 2 to 10 minutes

Prothrombin time 11 to 15 seconds

APTT 25 to 40 sec

Thrombin time 24 to 35 sec

Oral-B® at dentalcare.com Continuing Education Course, Revised March

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24, 2015
 RESULTS OF HEMOSTATIC SCREENING TEST
BLEEDING PLATELET PT APTT BT
COUNT
Thrombocytopenia, N N
leukemia
FVIII,IX,XI defecieny N N N
Heparin anticoagulant
FII,V,X, vit K def, intestinal N N
malabsorption
FVII def, coumarin N N N
anticoagulant, liver disease
Von willebrand disease N N N/

DIC, severe liver disease

FXIII defeciency N N N N

Vascular wall defect N N N

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BASIC MECHANISM OF HEMOSTASIS
Hemostasis can be divided into 4 general phases:
1. Vascular phase
2. Platelet phase
3. Coagulation cascade phase
• Intrinsic pathway
• Extrinsic pathway
• Common pathway
4. Fibrinolytic phase
The 1st 3 steps are the principal mechanisms that stop
the loss of blood following vascular injury.

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 CLOTTING FACTORS
•Factor I - fibrinogen
•Factor II - prothrombin
•Factor III - thromboplastin
•Factor IV - calcium
•Factor V - labile factor
•Factor VII - stable factor; serum prothrombin conversion accelerator (SPCA)
•Factor VIII - antihemophilic factor A
•Factor IX - christmas factor; plasma thromboplastin component; antihemophilic
factor B
•Factor X - stuart factor
•Factor XI - plasma thromboplastin anticedent (PTA); antihemophilic factor C
•Factor XII - hageman factor (contact factor)
•Factor XIII - fibrin stabilizing factor

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VESSEL WALL DISORDERS

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 SCURVY
 Dietary defeciency of water soluble vitamin C
results in scurvy. (dietary vitamin C below 10mg)
 Many of the hemorrhagic features of scurvy result
from defects in collagen synthesis. (vitamin C is
necessary for the synthesis of hydroxyproline, an
essential constituent of collagen).
 Recommended daily requirement of vit c is 30 to
70 mg.

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PATHOGENESIS:

•There is defective collagen formation in connective

tissue because of failure of hydroxylation of proline to
hydroxyproline which is a characteristic amino acid of
collagen.
•There is an increase in capillary permeability
(hemorrhage), and defective collagen formation.

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CLINICAL FEATURES:
•Lassitude
•Anorexia
•Pain in limbs
•Marked tendency to bleeding
•Hemorrhage may occur in the
joints
•Arrested skeletal development
•Enlargement of costochondral
junctions (scorbutic rosary)
•Delayed wound healing
•Anaemia
•Skin rashes
•Corkscrew hairs

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ORAL MANIFESTATIONS:
•Occurs chiefly in gingival and periodontal region
•Interdental and marginal gingiva appears bright red, swollen, smooth, shiny
surface producing an appearance known as scurvy bud.
•Colour changes to violaceous red
•Typical fetid breath
• In severe cases, gingiva becomes ulcerated and bleeds easily. There is
hemorrhage and swelling of periodontal ligament membrane followed by loss of
bone and loosening of teeth which are exfoliated.

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MANAGEMENT:

•Vitamin c supplementation –
•adult dosage is 800-1000 mg/day for at least one week,
then 400 mg/day until complete recovery.
•Children should be given 150-300 mg/day for one month.
•High vitamin C foods include bell peppers, green leafy
vegetables, kiwis, broccoli, berries, citrus fruits, tomatoes,
peas, and papayas. 

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HEREDITARY HEMORRHAGIC TELENGIECTASIA

•It is a genetically transmitted disease inherited as an autosomal

dominant trait.
•Also known as Osler–Weber–Rendu disease and Osler–Weber–
Rendu syndrome.
•There is abnormal blood vessel formation in the skin, mucous
membranes, and often in organs such as the lungs, liver, and
brain.
•Incidence : 1 in 5000
•Rarely found in children.

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CLINICAL FEATURES:
Characterized by multiple localized aneurysms on the finger tips, nose, face,
tongue and GI tract.
The lesion bleeds easily even after slightest trauma. Lesion blanches on
pressure
The typical lesion is cherry red to purplish macule or papule.
Epistaxis
Anemia

ORAL MANIFESTATIONS:
Severe oral hemorrhage . At times
there may be gush of blood when the
lesion is touched with cotton
Appears cherry red, often slightly
raised pinpoint lesion.

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DIAGNOSIS:
•Clinical diagnosis: cherry red lesion seen on mucosal site
•Laboratory diagnosis: intrinsic defect in the endothelial cells
permitting their detachment or defect in the perivascular
supportive tissue bed

MANAGEMENT:
•Septal dermoplasty: involved mucosa is removed and replaced by
skin graft.
•With regard to digestive tract lesions, mild bleeding and mild
resultant anemia is treated with iron supplementation.
•Pressure pack for spontaneous hemorrhage
•Sclerosing agents such as sodium morrhuate injected into the
lesion.
•Other therapies like estrogen containing creams or tranexamic
acid.
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PLATELET DISORDERS

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 WISKOTT-ALDRICH SYNDROME
• Wiskott–Aldrich syndrome  is a rare X-linked
recessive disease characterized
by eczema, thrombocytopenia (low platelet
count), immune deficiency, and bloody
diarrhea. 
• The estimated incidence of Wiskott-Aldrich
syndrome is between 1 and 10 cases per
million males worldwide; this condition is rarer
in females.
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Signs and symptoms
The disease occurs more oftenly in males.
Thrombocytopenia is present since birth.
The first signs of WAS are usually petechiae and bruising, resulting
from a low platelet count.
Spontaneous nose bleeds and bloody diarrhea are common. 
Eczema develops within the first month of life.
 Recurrent bacterial infections develop by three months. 
Enlargement of the spleen.
The majority of WAS children develop at least one autoimmune
disorder, and cancers (mainly lymphoma and leukemia) develop in
up to a third of patients.
IgM levels are reduced, IgA and IgE are elevated, and IgG levels can
be normal, reduced, or elevated.

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Treatment :based on correcting symptoms. 
•Aspirin and other nonsteroidal anti-inflammatory drugs should be
avoided, since these may interfere with platelet function.
•A protective helmet can protect children from bleeding into the
brain which could result from head injuries.
•For severely low platelet counts, patients may require platelet
transfusions or removal of the spleen.
•For patients with frequent infections, intravenous
immunoglobulins (IVIG) can be given to boost the immune system. 
•Anemia from bleeding may require iron supplementation or blood
transfusion.

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 GLANZMANN’S DISEASE
• Also called as ‘familial thrombasthenia’.
• It is hereditary, chronic hemorrhagic disease
transmitted as an autosomal recessive trait.
• platelets contain defective or low levels
of glycoprotein IIb/IIIa (GpIIb/IIIa), which is a
receptor for fibrinogen. As a result,
no fibrinogen bridging of platelets to other
platelets can occur, and the bleeding time is
significantly prolonged.
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CLINICAL FEATURES:
Severe bleeding either spontaneous or minor trauma
Purpuric hemorrhages of skin
Epistaxis and GI bleeding
Hematemesis noted in some cases

ORAL MANIFESTATIONS:
Spontaneous gingival bleeding

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DIAGNOSIS:
•Clinical diagnosis: severe excessive bleeding, epistaxis and haemarthrosis
•Laboratory diagnosis:
-bleedimg time is prolonged while clot retraction is impaired.
- Platelet count is normal. the aggregation of platelet by epinephrine , ADP
and thrombin is defective.

MANAGEMENT:
•Dental hygiene lessens gingival bleeding.
•Avoidance of antiplatelet agents such as aspirin and other (NSAIDs) and anticoagulants.
•Iron or folate supplementation may be necessary if excessive or prolonged bleeding has
caused anemia.
•Antifibrinolytic drugs such as tranexamic acid or ε-aminocaproic acid (Amicar).
•Desmopressin (DDAVP) does not normalize the bleeding time  but
improves hemostasis.
•Topical agents such as gelfoam, fibrin sealants, polyethylene glycol polymers.
•Platelet transfusions (only if bleeding is severe; risk of platelet alloimmunization).
•Recombinant factor VIIa .

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 AUTOIMMUNE OR IDIOPATHIC
THROMBOCYTOPENIC PURPURA
•It is characterized by immunologic destruction of
platelets and normal or increased megakaryocytes
in the bone marrow.
•On the basis of illness, ITP is classified into acute
and chronic forms.

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ACUTE ITP:
•This is a self limiting disorder, seen most frequently in children following recovery from a
viral illness or an upper respiratory illness.
•Onset is sudden and symptoms appear after 2 to 3 weeks of viral illness with sudden
onset of purpura and sometimes oral or nasal bleeding.
•Recovery occurs in 4 to 6 weeks.
•There is formation of immune complexes containing viral antigens and by formation of
antibodies against viral antigens which cross react with platelets and lead to their
immunologic destruction

CHRONIC ITP:
•Occurs more commonly in adults
•There is formation of antiplatelet autoantibodies
•These autoantibodies are directed against target antigens in the platelet glycoproteins,
GPIIb , IIIa.

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CLINICAL FEATURES:
Petechial hemorrhages
Easy bruising
Mucosal bleeding
Nasal bleeding
Bleeding from gums
Malaena
Haematuria
Splenomegaly
hepatomegaly Chronic ITP

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LABORATORY FINDINGS:
•Platelet count is reduced (10000-50,000/µl)
•Bone marrow shows increased megakaryocytes, indicating peripheral
destruction of platelets.
•With sensitivity testing, antiplatelet antibodies can be demonstrated on
platelet surface or the serum
•Platelet survival study shows reduced platelet life span.

TREATMENT:
Children:
•Prednisolone 2mg/kg orally. The platelet count rises in 1 to 3 days.
•Persistent bleeding or internal bleeding may be treated with platelet
transfusions.
•If bleedimg persists, iv immunoglobulins should be given.
•Adults:
•Prednisolone 60mg daily fro 2 to 4 weeks.
•Severe bleeding should be treated with platelet infusions. Iv immunoglobulin
should be given in a dose of 1g/kg if bleeding persists.

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DENTAL CONSIDERATIONS:
patients with ITP are at risk of prolonged or excessive bleeding.
A preoperative platelet level less than 20,000/cumm pose significant
risk of bleeding. So, dental treatment is done in a hospital with platelet
transfusion.
In patients with stable platelet count, dental surgerises are done with
precautionary hemostatic control measures.
Control of hemostasis at the site of surgery may be achieved by local
and systemic measures. When a transient increase in platelet count is
needed, iv IG- 1g/kg or oral corticosteroids is given the day before and
on the day of dental extraction or other procedures .
NSAIDs should be avoided .
Nerve blocks should be avoided. Infilteration, intrapulpal or
intraligamentary anesthesia is preferred.

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 THROMBOTIC THROMBOCYTOPENIC
PURPURA

•It is an uncommon but often fulminant and lethal

disorder occuring in young adults.
•caused by spontaneous aggregation of platelets and
activation of coagulation in the small blood vessels. 
•It is characterized by a triad of
•Thrombocytopenia
•Microangiopathic hemolytic anemia
•Formation of hyaline fibrin microthrombi
•A hereditary form of TTP is called the Upshaw Schulman
syndrome.
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CLINICAL FEATURES:
•Thrombocytopenia
•Hemolytic anemia
•Fever
•Neurologic symptoms (fluctuating), such as
hallucinations, bizarre behavior, altered mental status,
 stroke, or headaches.
•Renal failure

LABORATORY FINDINGS:
•Bone marrow examination reveals normal or slightly increased
megakaryocytes accompanied with some myeloid hyperplasia.

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TREATMENT:
• plasmapheresis has become the treatment of choice for TTP. This is
an exchange transfusion involving removal of the patient's blood
plasma  and replacement with donor plasma (fresh frozen plasma ); the
procedure must be repeated daily to eliminate the inhibitor and abate the
symptoms.
• Corticosteroids(prednisone or prednisolone) are usually given.
Rituximab, a monoclonal antibody aimed at the CD20 molecule on B
lymphocytes, may be used for diagnosis; this is thought to kill the B cells
and thereby reduce the production of the inhibitor.  A stronger
recommendation for rituximab exists where TTP does not respond to
corticosteroids and plasmapheresis.
•Children with Upshaw-Schülman syndrome receive prophylactic plasma
every two to three weeks; this maintains adequate levels of functioning
ADAMTS13. Some tolerate longer intervals between plasma infusions.
Additional plasma infusions may need to take place around triggering
events, such as surgery; alternatively, the platelet count may be
monitored closely around these events with plasma being administered if
the count drops. 54
 LEUKEMIA

Leukemia is a neoplastic proliferation of WBC in bone marrow,

usually in circulating blood and sometimes in other organs such
as liver, spleen and lymph nodes.

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CLASSIFICATION:
Leukaemias are classified on the basis of cell types into:
a. Myeloid
b. Lymphoid

On the basis of natural history of the disease :

c. Acute
d. chronic

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ETIOLOGY:
Ionising radiation
Cytotoxic drugs and chemical carcinogens
Inherited syndromes such as Downs syndrome
Occurs more commonly in whites
Epstein barr virus
Smoking
Immunological defeciency states

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 ACUTE LEUKEMIA

•Acute leukemias are characterized by predominance of

undifferentiated leucocyte precursors or leukemic blasts.
•Acute myeloblastic leukaemia is 4 times more common than acute
lymphoblastic leukaemia.

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 CLINICAL FEATURES:

More common between 15 to 39  Generalized swelling of

years of age lymph nodes
 Petechiae or
Males are more commonly
hemorrhage in skin and
affected than females (M:F- 3:2)
mucus membrane
Pyrexia  Bone pain and
Pallor, Anemia tenderness
Lethargy
 Vomiting
Dyspnoea
Headache  Nerve palsies

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ORAL MANIFESTATIONS:
Submental, submandibular lymph node enlargement
Paresthesia of lower lip and skin
Toothache due to leukaemic cell infilteration of dental pulp
Oral mucous membrane shows pallor, ulceration with necrosis,
petechiae, echymosis and bleeding tendency
Gingiva shows hypertrophy and cyanotic discoloration
Exudation from gingiva.

60
DIAGNOSIS:
Hb count, platelet count is low
Total WBC count is markedly
high
Bone marrow examination:
hypercellularity with leukemic
blast cells.
Erythropoietic and
megakaryocytic cells are reduced.
Peripheral smear shows
significant
number of immature granulocytes
or
lymphatic precursors or even
stem cells

61
MANAGEMENT:
1. Induction chemotherapy:
 
• vincristine (1.4mg i.v )

• prednisone(40mg orally )

• anthracycline

• cyclophosphamide (600 units) .

Using this approach, complete remissions (CRs) are obtained in 65-

85% of patients.

62
2. Consolidation chemotherapy:

• Daunorubicin i.v,

• mercaptopurine,

• methotrexate with intrathecal therapy using cytarabin and

methotrexate together with irradiation of cranium to eradicate the
disease from CNS.

3. Maintenance chemotherapy: patient receives a repeating cycle

of these drugs for 2 to 3 years.

63
4.TRANSPLANTATION: Allogenic bone marrow transplantation
from an identical twin.

5. SUPPORTIVE THERAPY: transfusion of red cells and platelets

may be required in cases of severe anemia and thrombocytopenia.

6. Topical treatment for gingival bleeding: direct pressure is

applied by use of absorbable gelatin or collagen sponge, topical
thrombin.

7. Management of oral ulcers: topical antibacterial with povidine

iodine solution, chlorhexidine rinses or tetracycline rinses.

64
 CHRONIC MYELOID LEUKEMIA

•It is a myeloproliferative disorder characterised by excessive

proliferation of myeloid series of cells in the marrow with fairly
normal maturation process.

•Cytogenic studies have shown that 90% patients of CML have a

chromosomal abnormality known as philadelphia chromosome

65
CLINICAL FEATURES:
Age: 35 to 60 years
Anaemia
Weight loss
Lassitude
Anorexia
Bone pain
Fever
Perspiration
Easy bruising
Epistaxis
Haematomas
splenomegaly

66
LABORATORY DIAGNOSIS:
Normocytic normochromic anaemia
Mean WBC count is markedly high( 50,000 – 5,00,000/ml)
Platelet count is often high
Blood film examination: myeloblasts are less than 10%. There is
an increase in absolute count of basophils, eosinophils and
nucleated red cells.

67
TREATMENT:
3 treatment modalities are available:

1. Drug therapy busalphan 2 – 4 mg orally or hydrxyurea ( preferred)

2 – 4 gm daily orally.

2. Alpha interferon therapy: advocated to induce and maintain

remission in chronic phase of the disease. It is given either i.m or
i.v in dosage of 3 to 9 mega units daily and the dosage should be
reduced.

3. Allogenic bone marrow transplantation

68
 CHRONIC LYMPHOCYTIC LEUKEMIA

•It is a slowly progressing malignancy involving lymphocytes.

•It is characterised by the accumulation of long lived, non-

functional B lymphocytes.

69
CLINICAL FEATURES:
Occurs more commonly in males and majority of patients are
above 45 years of age.

Pallor, weakness, dyspnoea, purpura(anemia)

Lymphadenopathy

Liver and spleen are enlarged and palpable.

Skin nodules, intestinal malabsorption, pulmonary obstruction

as a result of leukemic infiltration.

70
ORAL MANIFESTATIONS:
Hypertrophy of gingiva
Ulceration with necrosis and a gangrenous exudation
Dark brown exudate and foul fetor oris
Tongue is frequently swollen and dark
Regional lymphadenopathy
Loosening of teeth due to necrosis of periodontal ligament

71
LABORATORY FINDINGS:
Hb level is low
WBC count is as high as 1,50,000 to 2,00,000/μι
Platelet count is normal or reduced
Blood film shows 90 to 95% lymphocytes
Bone marrow shows abnormal proliferation of lymphocytes with
suppression of erythropoiesis
Immunoglobulin level is raised.

72
MANAGEMENT:

Supportive treatment:
maintain good health, adequate rest, good food and exercise.

Chemotherapy: chlorambucil 6 to 10 mg/day for 14 days

Combination therapy: cyclophosphamide doxorubicin, vincristine

and prednisone

Radiotherapy

Steroids: prednisone 40 mg daily.

73
74
 DENTAL CONSIDERATIONS IN
PATIENTS WITH LEUKEMIA

Sonis et al. proposed the classification of patients into

categories of high, moderate, and low risk for dental treatment,
based on the type of leukemia (acute or chronic) and
chemotherapy.

1. High risk
2. Moderate risk
3. Low risk

75
1. high-risk:

• active leukemia

• Thrombocytopenia

• Neutropenia

• patients under treatment for leukemia, and as a result of

therapy, present bone marrow suppression.

76
2. Moderate risk:
patients who successfully completed the first phase of treatment
(induction) and are undergoing the maintenance phase

3. Low-risk category:
are patients who successfully completed treatment and present no
evidence of malignancy or myelosuppression.

77
Little et al. and Elad et al. reinforce that the role of the dentist
should occur at three different moments:

(1)pre-antineoplastic treatment evaluation and patient preparation

(2)guidelines and oral health care during treatment

(3)Post treatment care.

78
1. Pre-Antineoplastic Treatment Assessment and Patient
Preparation

The dental examination should occur immediately after diagnosis and

before initiation of chemotherapy so as to permit the removal of
sources of infection of dental origin.

objectives

1)identify and eliminate sources of existing or potential infection.

(2)Oral hygiene instructions

(3)warn about the possible effects of antineoplastic therapy in the oral

cavity, such as mucositis;

79
AUTHORS PLATELET COUNT NEUTROPHIL COUNT

American >75,000 : without additional support.  >1,000 : no need for antibiotic

Academy of prophylaxis.
Pediatric 40,000 to 75,000 : Platelet transfusion
Dentistry, preoperative and postoperative <1,000 : Postpone the dental
2013 treatment. Hospitalization may be
<40,000 : Postpone the dental treatment. required.

US National >60,000 : without additional support. >2,000 : without the need for
Cancer antibiotic prophylaxis.
Institute, 30,000  to 60,000 : optional transfusion
2011 1,000 to 2,000 : antibiotic
<30,000 : Platelets should be transfused prophylaxis (low risk).
1 h before the procedure.
<1,000 : antibiotic prophylaxis with
Amikacin 150 mg/kg 1 h before
surgery and Ticarcillin 75 mg/Kg 
IV 1 h before surgery. Repeat both
6 h postoperative.

80
2. Oral Health Care during Antineoplastic Treatment

They are classified as high-risk patients

objectives :

(1)maintain optimal oral health

(2)treat side effects of antineoplastic therapy

(3)reinforce to the patient the importance of oral health in reducing

problems/discomforts arising from chemotherapy.

81
AUTHORS PLATELET COUNT NEUTROPHIL COUNT

Sonis et al., <100,000 : elective dental treatment <3,500  (leukocytes):

1995  should be postponed. elective dental treatment
should be postponed.

Brennan et <50,000 : contraindication to perform <1,000 : contraindication to

al., 2008  invasive procedures. perform invasive
procedures.

82
3. Post-Antineoplastic Treatment Oral Health Care

patients are considered cured of leukemia and not having oral

manifestations with the exception of those with sequelae of
radiotherapy or children who received chemotherapy in the stage of
tooth formation , which may present hypoplastic areas on tooth
enamel (mineralization disorder) and changes in the development of
dental roots

83
 APLASTIC ANEMIA

• Aplastic anemia is a bone marrow failure syndrome

characterised by peripheral pancytopenia and general lack of
bone marrow activity.

• There are 2 chief forms of aplastic anemia

-Primary aplastic anemia

-secondary aplastic anemia

84
Primary aplastic anemia :

• is a disease of unknown etiology.

•Develops more frequently in young adults.

•Develops rapidly and usually terminates fatally.

85
Secondary aplastic anemia: known etiology.

•Occurs at any age

• chemicals such as acetophenitidine, organic arsenicals, benzol,

colloidal silver, mercury, bismuth

•Radiant energy in the form of x-rays, radium or radioactive

isotopes.

•Preceeded by infections by EBV, hepatitis virus, HIV.

86
CLINICAL FEATURES:
The onset is insiduous, with initial symptom relating to anemia or
bleeding

Leukopenia

Thrombocytopenia

Weakness

Dyspnoea

Numbness and tingling of the extremities

87
Petechiae of the skin and mucus membrane

Rapid or irregular heart rate

Pale skin

Frequent or prolonged infections

Unexplained or easy bruising, Prolonged bleeding from cuts

 Skin rash

Dizziness

Headache
88
ORAL MANIFESTATIONS:
Peteciae, purpuric spots or frank hematomas of oral mucosa

Spontaneous gingival hemorrhage

Ulcerative lesions of the oral mucosa or pharynx

89
LABORATORY FINDINGS:

RBC count is as low as 1,00,000 cells/ cu mm

Prolonged bleeding time

Clotting time is normal

Anemia is normocytic with some degree of macrocytosis.

90
MANAGEMENT:

Removal of cause

Supportive therapy: antibiotics and transfusions

Bone marrow transplantation

Antifibrinolytic agents such as aminocaproic acid

91
 ANTICOAGULANT-RELATED
COAGULOPATHIES
HEPARIN
•Heparin is a potent anticoagulant that binds with antithrombin III
to dramatically inhibit activation of Fs IX, X, and XI, thereby
reducing thrombin generation and fibrin formation.

•The major bleeding complications from heparin therapy are

bleeding at surgical sites and bleeding into the retroperitoneum.

•Heparin has a relatively short duration of action of 3 to 4 hours,

so is typically used for acute anticoagulation, whereas chronic
therapy is initiated with coumarin drugs.

• Protamine sulfate can rapidly reverse the anticoagulant effects

of heparin 92
Coumarin

•Coumarin anticoagulants, which include warfarin and

dicumarol, are used for anticoagulation to prevent recurrent
thrombotic phenomena (pulmonary embolism, venous
thrombosis, stroke, myocardial infarction), to treat atrial
fibrillation, and in conjunction with prosthetic heart valves.

93
•They slow thrombin production and clot formation by blocking the
action of vitamin K. Levels of vitamin K–dependent Fs II, VI, IX,
and X (prothrombin complex proteins) are reduced.

• The anticoagulant effect of coumarin drugs may be reversed rapidly

by infusion of fresh frozen plasma, or over the course of 12 to 24
hours by administration of vitamin k.

94
 MAY- HEGGLIN ANOMALY

• is a rare genetic disorder of the blood platelets

•also known as Dohle leukocyte inclusions with giant platelets

and macrothrombocytopenia with leukocyte inclusions.

•Patients are often asymptomatic. The bleeding tendency

associated with MHA is generally mild 

95
COAGULATION DISORDERS

96
 VON WILLEBRAND’S DISEASE
It is the most common hereditary coagulation disorder occuring
due to qualitative or quantitative defect in von willebrand’s
factor.
Incidence : 1 in 1000individuals.
CLINICAL FEATURES:
Clinically, the patients of vWD are characterised by spontaneous
bleeding from mucous membranes and excessive bleeding from
wounds.

97
There are 3 major types:

Type I disease: is the most common and is characterised by mild

to moderate decrease in plasma vWF. The synthesis of vWF is
normal but the release of its multimers is inhibited.

Type II disease: much less common and is characterised by

normal or near normal levels of vWF which is functionally
defective.

Type III disease: extremely rare and is the most severe form of
the disease. These patients have no detectable vWF activity and
may have sufficiently low factor VIII levels.

98
Laboratory findings:
• Prolonged bleeding time

• Reduced plasma vWF concentration

• Defective platelet aggregation

• Reduced factor VIII activity

TREATMENT: bleeding episodes in vWD are treated with

cryoprecipitates or factor VIII concentrates

99
 HEMOPHILIA

• Hemophilia is a blood disease characterized by a prolonged

coagulation time and bleeding tendencies.

• The disease is hereditary, the defect being carried by the X

chromosome, and is transmitted as gender linked mendelian
recessive trait; thus hemophilia occurs only in males.

• Haemophilia is rare, with only about 1 instance in every

10,000 births (or 1 in 5,000 male births) for haemophilia A and
1 in 50,000 births for haemophilia B

100
 HISTORY

• The first medical professional to

describe a disease was Abulcasis. In the
tenth century, he described families whose
males died of bleeding after only minor traumas.

• Haemophilia has featured prominently in European

royalty and thus is sometimes known as 'the royal disease'.
Queen Victoria passed the mutation for Haemophilia B  to her
son and, through some of her daughters, to various royals
across the continent.

101
102
 HEMOPHILIA A

• It is an inherited disorder of factor VIII defeciency.

• 2nd most common disorder of coagulation system.

• The disorder is inherited as X-linked recessive trait .

• Manifests clinically in males while females are carriers.

103
CLINICAL FEATURES:
 bleeding into soft tissues, muscles and weight bearing joints.

hemarthosis which produces pain, swelling and tenderness of

joints.

disorganisation of joints.

The most frequent sites for bleeding are the knees, followed by the
elbows, ankles, hips and wrists

Muscle hematomas

Haematuria

104
ORAL MANIFESTATIONS:
Common sites involved are the frenum and tongue

Prolonged bleeding after tooth extraction

Hematoma of the floor of the mouth

Physiological processes of tooth eruption

and exfoliation may be associated with severe and prolonged
hemorrhage.

Gingival hemorrhage as a result of gingival injury.

105
106
 HEMOPHILIA B

• It is an X linked inherited disease caused due to reduction in

plasma factor IX level.

• Less common than hemophilia A.

• It is also called christmas disease.

• It is diagnosed by a positive family history, a history of

bleeding episodes and a prolonged aPTT test with a normal
PT, along with inadequate levels of factor IX.

107
 Classification of Degree of Risk of a Bleed After Trauma or Surgery
Hemophilia A and B Deficiency

Delayed onset of a bleed with trauma or

Mild 5% to 30%
surgery or dental extractions

Moderate 1% to 5% Excessive bleeding with surgery

Severe Less than 1% Excessive bleeding with trauma or surgery

108
TREATMENT:

Management of hemostasis

Use of factor replacement products and medication

Oral bleeding from the frenum and after tooth extraction

combine adequate replacemnt therapy with an antifibrinolytic
agent (E-aminocaoroic acid, tranexamic acid) to neutralize the
fibrinolytic activity in the oral cavity.

Topical agents such as fibrin sealant, bovine thrombin and

human recombinant thrombin, gelfoam etc can be used.

109
Hematoma in the pharynx or epiglottic region frequently
results in partial or complete airway obstruction; therefore it
should be treated with aggressive infusion therapy.

Administer prophylactic infusion therapy before an oral

surgical procedure.

 The patient will need to be hospitalized . Nerve blocks should

be given only after fctor VIII infusion for hemophilia A and
factor IX nfusion for hemophilia B.

110
 SOURCES CONCENTRATION OF FACTOR VIII

Fresh frozen plasma (blood bank) 1 unit/ml

cryoprecipitate 5 to 10 units/ml

Plasma derived lyophilized factor VIII 250 to 500 units/vial

concentrate( commercially available)

Genetically engineered factor VIII 250 to 500 units/vial

(commercially available)

111
112
DENTAL CONSIDERATIONS IN PATIENTS
WITH HEMOPHILIA
Treatment Planning Considerations

Step 1:Patient and Clinical Assessment

•systematic approach of collecting, organizing, and evaluating
all patient data. 

• Data must be retrieved from the personal, medical and dental

histories, the pharmacological history, laboratory testing, and
inspection of the extra and intraoral structures. 

113
Step 2: 
•Consult with the supervising physician to obtain additional
information about the patient’s disorder or bleeding history.
 
•obtain medical clearance to treat. 

•retrieve and evaluate the blood laboratory test results while

scheduling the appointment within 24 hours of the results.

step 3: 
Develop an appropriate treatment plan: establish whether or not
the invasive dental procedure will be carried out in the dental
office or in a hospital-based dental facility.

114
Pre-treatment of invasive procedure: Management recommendations:

Hemophilia A: Factor VIII

Consult with hematologist replacement, desmopressin (increases
factor level)

Confirm diagnosis and severity of

e-aminocaproci acid (stablizes the clot)
hemophilia

Patients with mild to moderate

Hemophilia B: Purified Factor IX
hemophilia are usually treated in the
products
dental office

von Willebrand’s: Factor VIII

Patients with severe hemophilia are
replacement; vWF in some cases; and
usually treated in a dental-based hospital
Hemophilia A management
setting
recommendation

115
 Management during invasive Management after the procedure:
procedure: Monitor bleeding:

Hospitalize the pt if bleeding is not

Use good surgical technique
controlled

Examine pt 24-48 hrs post procedure:

Use hemostatic agents
treat infection and/or bleeding issues

Hematologist will monitor hospitalized Avoid aspirin, use acetaminophen with

patient or without codeine

116
Drug Products and Dental Procedures Used as Local Measures to Limit and
Control Bleeding During Invasive Dental Procedures
BRAND CHEMICAL MECHANISM OF ACTION CONTRAINDICATIO DISADVANT
NAME NAME N AGE

Gauze 2”X2” sterile gauze pads;

place pressure on wound
or apply finger prssure

Gelfoam Absorbable gelatin sponge Should not be used

material; provides stable under epithelial
‘scaffold ‘ for clot incisions or flaps,
formation inhibits healing

Surgicel Oxidized regenerated

cellulose, exerts physical
effect rather than
physiological

Bleed X Hemostatic product

containing micropores ;
dehydrates blood and
accelerates clotting

117
Tissel Fibrin sealant, adhesive Technique sensitive,
action that binds fibrin to requires special
the clot attention to
preparation
Cyclopan Tranexamic Used in the form of a
acid mouthwash after surgical
procedures to inhibit
postoperative bleeding,
can be used parenterally
or as 4.8% aqueous
solution
Suturing Apposition of tissues

Amicar Aminocapr Antifibrinolytic agent No longer used

oic acid

Electrocau Tool to slow

tery intraoperative bleeding
and interfere with postop
bleeding episodes.

118
 Presurgery treatment for hemophilia A

Condition Treatment and dose Potential complications

Mild bleeding Dose: 15 U/kg factor VIII Hemarthrosis, oropharyngeal

every 8–12 hours for 1–2 or dental bleeding, epistaxis,
days hematuria

Major bleeding Dose: 50 U/kg factor VIII Same potential complications

every 8–12 hours for 7–14 as for mild bleeding, as well as
days central nervous system
hemorrhage, retroperitoneal
hemorrhage, gastrointestinal
bleeding

119
  The severity of haemophilia, clinical manifestations and
recommendations for dental treatments

Degree of Factor Clinical

haemophili percen Dental treatment
features
a tage

Frequent Enhanced preventive advice.

Severe <1% spontaneous Should have all dental treatments except for
bleeds prosthetics carried out in a hospital setting

May have Enhanced preventive advice and treatment with

Moderate 2-5% spontaneous general dental practitioner.
bleeds Manage as for severe haemophilia.

Bleed after
Enhanced preventive advice. Do not require all
Mild 6-40% trauma or
treatments carried out at the hospital.
surgery

Guidance on the dental management of patients with haemophilia and congenital bleeding
disorders 22 November 2013
120
PRECAUTIONS TO BE TAKEN DURING A DENTAL
TREATMENT:

PAIN CONTROL:

•nerve-block anesthetic injections are contraindicated Anesthetic .

•An anesthetic with a vasoconstrictor should be used.

•Alternative techniques, including sedation with diazepam or nitrous

oxide, can be employed to reduce or eliminate the need for anesthesia.

121
ORAL SURGICAL PROCEDURES:

• local hemostatic agents and techniques such as pressure, surgical

packs, oxidized celulose, topical thrombin .

•After packing, the socket must be protected by a stent to prevent

disturbance to the clot.

122
Periodontal Procedures:

•Factor replacement is needed for subgingival scaling and root

planing and extensive periodontal surgery.

• For severely inflamed tissues, initial treatment with chlorhexidine

mouthwashes and gross debridement is recommended.

•Periodontal packing materials and custom vinyl mouthguards

(stents) are used to aid in hemostasis and protect the surgical site .

•Antifibrinolytic agents may be incorporated into periodontal

dressings for enhanced effect.

• Post-treatment antifibrinolytic mouthwashes are usually effective

in controlling protracted bleeding.
123
Restorative and Endodontic Procedures:

•Care should be taken to avoid injuring the gingiva while placing

rubber dam clamps, matrices and wedges.

•A rubber dam should be used to prevent laceration of soft tissues

by the cutting instruments. Saliva ejectors and high-speed suction
they should be used carefully.

• Endodontic therapy is preferred over extraction whenever

possible.

•Care should be taken not to exceed beyond the apex of the tooth.

124
PROSTHODONTIC THERAPY:
Trauma should be minimized by careful post-insertion adjustments.

 Orthodontic Procedures:
Orthodontic therapy can be carried out without bleeding
complications, although care should be taken that appliances do not
impinge on soft tissues and emphasis should be put on excellent,
atraumatic oral hygiene.

125
 HOME REMEDIES TO CONTROL
BLEEDING
1.Coffee Powder – the best way to stop a
bleeding cut is to immediately wash the wound
and apply coffee powder to it. The use of coffee
powder on the wound will help to clot the blood
thus reducing the bleeding.

2.Flour –when you get a minor cut, applying

Maida or wheat flour to the wound will stop the
bleeding in seconds.

126
3.Electrical Tape :The use of electrical
tape is considered to stop the bleeding. The
Electrical tape has to be tied near the
wound so that the blood flow stops thus
leaving you with no scars.

4.Salt :salt powder mixed with warm water

can not only heal wounds but also act as a
home remedies to stop bleeding.

127
5. Use an ice pack. Fill an ice
pack or cold compress. Wrap it
in a towel and place it over the
wound. The cold can constrict
blood vessels, which will help
stop the bleeding. It can also
numb the area, reducing pain for
the injured person. Never place
ice, an ice pack, or cold pack
directly on a wound. The
extreme cold can damage the
skin tissue.

128
6. Clove Oil: Considered to be one
of the best home remedies, clove
oil helps to reduce the
inflammation of gums and stops the
gums from bleeding to a larger
extent. Take a little clove oil and
rub it on your gums or chew one or
two cloves.

129
7. Use Aloevera: Aloevera has
many medicinal properties and
one of the properties is to
reduce gum inflammation,
making bleeding less likely.
Take a small amount of
aloevera pulp and massage it
on your gums, Let the pulp
settle on your gums before
rinsing your mouth.

130
 FACTOR XI DEFICIENCY
•It is the least common type of hemophilia. It is found in
2-3% of all hemophiliac patients.

•Plasma thromboplastin antecedent deficiency is clinically

a mild disorder seen in pedigrees of Jewish descent;

•it is transmitted as an autosomal dominant trait. Bleeding

symptoms do occur but are usually mild.

•In the event of major surgery or trauma, hemorrhage can

be controlled with infusions of fresh frozen plasma.
 FACTOR XII DEFICIENCY
•Hageman factor deficiency is another rare disease that
presents in the laboratory with prolonged PT and partial
thromboplastin time (PTT).

•Clinical symptoms are nonexistent. Treatment is therefore

contraindicated.
FACTOR X DEFICIENCY
• Stuart factor deficiency, also a rare bleeding diathesis, is
inherited as an autosomal recessive trait.

•Clinical bleeding symptoms in the patient with levels less

than 1% are similar to those seen in hemophilias A and B.
 FACTOR V DEFICIENCY
Proaccelerin deficiency, is a rare autosomal recessive trait that
presents with moderate to severe clinical symptoms.

When compared with hemophilias A and B, this hemorrhagic

diathesis is moderate, only occasionally resulting in soft-tissue
hemorrhage, and only rarely presenting with hemarthrosis;

it does not involve the devastating degenerative joint disease

seen in severe hemophilias A and B.
 FACTORS XIII AND I DEFICIENCIES
•Fibrin-stabilizing factor deficiency and fibrinogen
deficiency are very rare, and these diagnosis can be made only
with extensive laboratory tests usually available only in tertiary-
care medical centers. Both are autosomal recessive traits.

• Most dysfibrinogenemias result in no symptoms, others lead

to moderate bleeding, and a few induce a hypercoagulable state.
Factor XIII deficiency appears to have different forms of
penetrance, and in some families appears only in the males.
 VITAMIN K DEFECIENCY
•Vitamin K is a necessary cofactor for carboxylation of factors II,
VII, IX and X.
•Vitamin k defeciency results in abnormal blood coagulation and
bleeding.
•Vitamin k defeciency occurs in biliary obstuction, malabsorption
syndrome, prolonged antibiotic therapy, nutritional defeciency and
warfarin ingestion.
•Prothrombin time is usually prolonged.

136
•Treatment:

•Administration of vit k 10mg for 1-3 days.

•Patients with severe hemorrhage due to vit k defeciency can be

managed by transfusing fresh frozen plasma.

137
FIBRINOLYTIC DISORDERS

138
 DISSEMINATED INTRAVASCULAR
COAGULATION
• It is an acquired bleeding disorder.

• Also termed as defibrination or consumption coagulopathy.

• It may be acute, subacute or chronic.

139
CLINICAL FEATURES:

•Skin and mucous membrane bleeding

•Hemorrhage from multiple sites

•Thrombosis. (rare)

•Pregangrenous changes in digits, genetalia and nose.

140
LABORATORY FINDINGS:

•Platelet count is low

•Prothrombin time and thrombin time is prolonged.

•Plasma fibrinogen level is low.

MANAGEMENT:

•Fresh frozen plasma, red cell concentrates and cryoprecipitate to

replace depleted clotting factors.

•Platelet concentrates to correct thrombocytopenia.

141
 CONCLUSION
• As health care providers we are ethically and legally obligated to
provide the highest standards of care for our dental patients.

• Knowledge of basic physiological events of hemostasis and the

pathophysiological events associated with hemorrhagic disorders is
important for proper treatment planning and dental management of such
patients.

• Comprehensive assessment of data, including laboratory tests, diagnosis

of the condition , individualised treatment planning ,with regard to
controlling the bleed and careful manipulation of tissues during
implementation is important for successful management of dental
patients with bleeding disorders.

142
 REFERENCES
ESSENTIAL PATHOLOGY FOR DENTAL STUDENTS BY HARSH
MOHAN- 3RD EDITION.

SHAFER’S TEXT BOOK OF ORAL PATHOLOGY – 6TH EDITION

DE GRUCHY’S CLINICAL HAEMATOLOGY IN MEDICAL PRACTICE-

5TH EDITION EDITED BY FRANK FIRKIN, COLIN CHESTERMAN,
PENINGTON AND BRYAN RUSH

ESSENTIALS OF MEDICAL PHYSIOLOGY- K SEMBULINGAM,

PREMA SEMBULINGAM- 4TH EDITION

TEXTBOOK OF MEDICAL PHYSIOLOGY- GUYTON AND HALL- 10TH

EDITION.

143
MANUAL OF PRACTICAL PHYSIOLOGY- AK JAIN

TEXTBOOK OF ORAL MEDICINE AND PATHOLOGY ;BURKITTS 10TH

EDITION

TEXTBOOK OF ORAL MEDICINE ANIL GOVINDRAO GHOM- 2ND

EDITION

TEXTBOOK OF CLINICAL MEDICINE SN CHUG.

TEXBOOK OF ORAL AND MAXILLOFACIAL SURGERY – CHITRA

CHAKRAVARTHY- 2ND EDITION

TEXTBOOK OF ORAL AND MAXILLOFACIAL SURGERY NEELIMA

ANIL MALIK- 3RD EDITION

MEDSCAPE- EDWARD CHARBEK, SHERILYN ALVARAN TUAZON

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Dental Management of Patients with Bleeding Disorders BY Sandra
D’Amato-Palumbo Revised March 24, 2015.

IDIOPATHIC THROMBOCYTOPENIC PURPURA , OVERVIEW WITH

REPORT OF A CASE BY SUNITHA M, R RAJESH

Bleeding Disorders of Importance in Dental Care and Related Patient

Management Anurag Gupta, BDS; Joel B. Epstein, DMD, MSD, FRCD(C);
Robert J. Cabay, MD, DDS February 2007

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THANK YOU………..

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