Blood coagulation disordersBlood coagulation disorders

Dr. Klara VezendiDr. Klara Vezendi

Szeged UniversitySzeged University

Transfusiology DepartmentTransfusiology Department

The normal haemostasis prevents:The normal haemostasis prevents:

●● spontaneous haemorrhage and undue blood loss spontaneous haemorrhage and undue blood loss from injured vessels from injured vessels

●● intravascular intravascular thrombus formation.thrombus formation.

bleedingbleeding thrombosisthrombosis

There are three components of blood coagulation There are three components of blood coagulation system:system:

HAEMOSTASISHAEMOSTASIS

1. 1. CapillariesCapillaries

2. 2. Platelets Platelets

3. 3. Plasma Plasma

coagulation coagulation factorsfactors

1. 2: Primary haemostasis 1. 2: Primary haemostasis (it is enough to stop bleeding from (it is enough to stop bleeding from small injuries) small injuries)

3: Secundary haemostasis 3: Secundary haemostasis (it is necessary to stop bleeding (it is necessary to stop bleeding definitely) definitely)

Primary haemostasis I:Primary haemostasis I:

● ● CapillariesCapillaries and larger blood vessels react to injury by and larger blood vessels react to injury by an immediate local temporary an immediate local temporary vasoconstrictionvasoconstriction (a reflex nervous (a reflex nervous mechanism) to reduce the amount of blood lost.mechanism) to reduce the amount of blood lost.

Primary haemostasis II:Primary haemostasis II:

● ● Platelets:Platelets:

- - adhereadhere to the site of injury to the site of injury

- - aggregationaggregation

- - release release substances from their cytoplasms to initiate substances from their cytoplasms to initiate blood coagulation blood coagulation haemostatic plug is formed.haemostatic plug is formed.

Secundary haemostasis:Secundary haemostasis:Blood coagulation factorsBlood coagulation factors are necessary to stop bleeding are necessary to stop bleeding

definitely. definitely.• I: fibrinogenI: fibrinogen• II: prothrombinII: prothrombin• III: tissue thromboplastin (tissue factor, TF)III: tissue thromboplastin (tissue factor, TF)• IV: CaIV: Ca++++ • V: proaccelerinV: proaccelerin• VI: -VI: -• VII: proconvertinVII: proconvertin• VIII: antihemophilic factor (AHF)VIII: antihemophilic factor (AHF)• IX: Christmas factor (plasma thromboplastin component)IX: Christmas factor (plasma thromboplastin component)• X: Stuart factorX: Stuart factor• XI: plasma thromboplastin antecedent (PTA)XI: plasma thromboplastin antecedent (PTA)• XII: Hageman factor (contact factor)XII: Hageman factor (contact factor)• XIII: fibrin stabilizing factor (Laki-Lorand factor)XIII: fibrin stabilizing factor (Laki-Lorand factor)

Disorders of the haemostatic mechanism areDisorders of the haemostatic mechanism are devided into three main groups:

• Disorders of the vesselsDisorders of the vessels

• Disorders of the plateletsDisorders of the platelets

• Disorders of the coagulation mechanism Disorders of the coagulation mechanism („coagulopathies”)(„coagulopathies”)

„ purpuric purpuric diseases”diseases”

The investigation of a patient with a The investigation of a patient with a suspected disorder of haemostasissuspected disorder of haemostasis

– case history (personal details, family case history (personal details, family history)history)

– inspection (type of bleeding)inspection (type of bleeding)

– physical examinationphysical examination

– other known diseasesother known diseases

– drugs and medicationsdrugs and medications

– laboratory testslaboratory tests

Certain signs and symptoms are virtually diagnostic of Certain signs and symptoms are virtually diagnostic of disordered haemostasis.disordered haemostasis.

The main symptom of all diseases is the The main symptom of all diseases is the bleeding:bleeding:

● ● in the in the „purpuric disorders”„purpuric disorders” cutaneous and mucosal cutaneous and mucosal bleeding usually is prominentbleeding usually is prominent

● ● in different types of in different types of „coagulopathies”„coagulopathies” hemarthroses, hemarthroses, haematomas are the characteristic bleeding manifestations. haematomas are the characteristic bleeding manifestations.

The onset of bleeding following trauma frequently is The onset of bleeding following trauma frequently is delayeddelayed (recur in a matter of hours) (recur in a matter of hours) (the temporary hemostatic adequacy of the (the temporary hemostatic adequacy of the platelet platelet plug may explain this phenomenon).plug may explain this phenomenon).

Petechiae, purpuras: Petechiae, purpuras: small capillary haemorrhages ranging from the size of a pinhead to much largersmall capillary haemorrhages ranging from the size of a pinhead to much larger

Petechiae, purpurasPetechiae, purpuras

Haematomas: Haematomas: may be spontaneous (in a serious hemorrhagic disease) or may occur may be spontaneous (in a serious hemorrhagic disease) or may occur after trauma (in a mild hemorrhagic disease).after trauma (in a mild hemorrhagic disease).

HaematomasHaematomas

Intramuscular injection may be very dangerous to Intramuscular injection may be very dangerous to the patient with a bleeding disorderthe patient with a bleeding disorder

Venipuncture (if skilfully Venipuncture (if skilfully performed) is without danger performed) is without danger becouse the elasticity of the venous becouse the elasticity of the venous walls.walls.

Screening tests of blood coagulation Screening tests of blood coagulation • Disorders of vessels:Disorders of vessels:

– Rumpel-Leede testRumpel-Leede test

• Disorders of platelets:Disorders of platelets:– Platelet count and morphologyPlatelet count and morphology

– Bleeding time (Ivy)Bleeding time (Ivy)

• Coagulopathies:Coagulopathies:– Coagulation timeCoagulation time

– Aktivated partial thromboplastin Aktivated partial thromboplastin time (APTT)time (APTT)

– Prothrombin (INR)Prothrombin (INR)

– Thrombin time (TT)Thrombin time (TT)

Laboratory diagnosis of the coagulopathiesLaboratory diagnosis of the coagulopathies

Contact activationContact activation Tissue thromboplastin (TF)Tissue thromboplastin (TF)

XIIXII

XIXI

IXIX

VIIIVIII

VIIVII

XX

VV

IIII

II

INTRINSICINTRINSIC EXTRINSICEXTRINSIC

COMMONCOMMON

Blood Blood coagulation coagulation timetime

APTIAPTI

Prothrom-Prothrom-binbin

FibrinFibrin

Platelet Platelet countcount

Bleeding Bleeding timetime

APTIAPTI Prothrom-Prothrom-binbin

Presumptive Presumptive diagnosisdiagnosis

DecreasedDecreased ProlongedProlonged Norm.Norm. Norm.Norm. ThrombocytopeniaThrombocytopenia

Norm.Norm. ProlongedProlonged ProlongedProlonged Norm.Norm. von Willebrand’s diseasevon Willebrand’s disease

Norm./Norm./ increasedincreased

ProlongedProlonged Norm. Norm. Norm.Norm. ThrombocytopathiaThrombocytopathia

Norm.Norm. Norm. Norm. ProlongedProlonged Norm.Norm. „„intrinsic” pathway intrinsic” pathway abnormality abnormality (FVIII. IX. XI. XII)(FVIII. IX. XI. XII)

Norm.Norm. Norm. Norm. Norm. Norm. ProlongedProlonged „„extrinsic”pathway extrinsic”pathway abnormality abnormality (FVII)(FVII)

Norm.Norm. Norm.Norm. ProlongedProlonged ProlongedProlonged „„common” pathway common” pathway abnorm. abnorm. (FI. II. V. X.)(FI. II. V. X.)

Norm.Norm. Norm.Norm. Norm.Norm. Norm.Norm. - /FXIII deficiency/ milde - /FXIII deficiency/ milde bleeding disorderbleeding disorder

Diagnosis of bleeding disorders by the screening testsDiagnosis of bleeding disorders by the screening tests

CoagulopathiesCoagulopathies• Aquired:Aquired:

generally generally several coagulation several coagulation abnormalities abnormalities are present. are present. Clinical picture is complicated Clinical picture is complicated by signs and symptoms of by signs and symptoms of the the underlying diseaseunderlying disease.. Deficiencies of the vitamin K Deficiencies of the vitamin K dependent coagulation factors (FII, dependent coagulation factors (FII, VII, IX, X) VII, IX, X) Hepatic disorders Hepatic disorders Accelerated destruction of blood Accelerated destruction of blood coagulation (DIC) coagulation (DIC) Inhibitors of coagulation Inhibitors of coagulation Others (massive transfusion, Others (massive transfusion, extracorporal circulation)extracorporal circulation)

• Hereditary:Hereditary: deficiency or abnormality of deficiency or abnormality of a a single coagulation factor.single coagulation factor.

– Hemofilia A (FVIII)Hemofilia A (FVIII)

– Hemofilia B (FIX)Hemofilia B (FIX)

– Von Willebrand’s diseaseVon Willebrand’s disease

– Rare coagulopathies Rare coagulopathies (FI. II. V. VII. X. XI. XIII)(FI. II. V. VII. X. XI. XIII)

HaemophiliaHaemophiliaA bleeding disorder in which clotting factor VIII A bleeding disorder in which clotting factor VIII (eight) (eight) /Haemophilia A/ /Haemophilia A/ or IX (nine) or IX (nine) /Haemophilia B//Haemophilia B/ in a person's blood plasma is missing or is at a low in a person's blood plasma is missing or is at a low level. level.

Prevalence:Prevalence:haemophilia A: 105/million menhaemophilia A: 105/million menhaemophilia B: 28/million menhaemophilia B: 28/million men

• In haemophilia, VIII In haemophilia, VIII or IX clotting factor is or IX clotting factor is missing, or the level of missing, or the level of that factor is low. that factor is low.

• This makes it This makes it difficult for the blood difficult for the blood to form a clot, so to form a clot, so bleeding continues bleeding continues longer than usual.longer than usual.

The hemophilia gene is carried on the The hemophilia gene is carried on the X chromosome X chromosome in males who lack a normal allele, the defect is manifested by in males who lack a normal allele, the defect is manifested by clinical haemophilia. Women may be carriers.clinical haemophilia. Women may be carriers.

Haemophilia is a Haemophilia is a lifelong diseaselifelong disease

• A person born with A person born with haemophilia will have haemophilia will have it for life. it for life.

• The level of factor VIII The level of factor VIII or IX in his blood or IX in his blood usually stays the same usually stays the same throughout his life. throughout his life.

Clinical manifestationsClinical manifestations

The most dramatic manifestation of haemophilia is The most dramatic manifestation of haemophilia is extensive extensive bleedingbleeding into the soft tissue and muscles into the soft tissue and muscles after after only negligible trauma, or even no known trauma.only negligible trauma, or even no known trauma.

The frequency and severity of bleeding generally is The frequency and severity of bleeding generally is related to the blood level of FVIII or FIX. related to the blood level of FVIII or FIX.

Haemophilia can be mild, moderate, or severe, Haemophilia can be mild, moderate, or severe, depending on the level of clotting factor. depending on the level of clotting factor.

Three category of severity:Three category of severity:

• Severe:Severe: FVIII/FIX FVIII/FIX < 1 %< 1 %– Repeated and severe hemarthroses and spontaneous Repeated and severe hemarthroses and spontaneous

bleeding, crippling common.bleeding, crippling common.

• Moderate:Moderate: FVIII/FIX: FVIII/FIX: 1-5 %1-5 %– Spontaneous bleeding and hemarthroses infrequent. Spontaneous bleeding and hemarthroses infrequent.

Serious bleeding from trivial injuries.Serious bleeding from trivial injuries.

• Milde:Milde: FVIII/FIX: FVIII/FIX: 5-40 %5-40 %– Spontaneous bleeding manifestations may be absent, Spontaneous bleeding manifestations may be absent,

although serious bleeding may follow surgical although serious bleeding may follow surgical procedures or traumatic injury. procedures or traumatic injury.

Joint bleedingJoint bleeding

As blood fills the capsule, the joint As blood fills the capsule, the joint swells and becomes painful and hard to swells and becomes painful and hard to move. move.

The most common joint bleeds happen The most common joint bleeds happen in ankles, knees, and elbows. in ankles, knees, and elbows. Bleeds into other joints can also happen. Bleeds into other joints can also happen.

The long-term effects of joint The long-term effects of joint bleeds:bleeds:Repeated bleeding into a joint causes the Repeated bleeding into a joint causes the synovium to swell and bleed very easily. synovium to swell and bleed very easily. Some blood remains in the joint after each Some blood remains in the joint after each bleed. The synovium stops producing the bleed. The synovium stops producing the slippery, oily fluid that helps the joint move. slippery, oily fluid that helps the joint move. This damages the smooth cartilage that This damages the smooth cartilage that covers the ends of the bones. The joint covers the ends of the bones. The joint becomes stiff, painful to move, and unstable. becomes stiff, painful to move, and unstable. It becomes more unstable as muscles around It becomes more unstable as muscles around the joint weaken. the joint weaken.

With time, most of the cartilage breaks down With time, most of the cartilage breaks down and some bone wears away. Sometimes the and some bone wears away. Sometimes the joint cannot move at all. joint cannot move at all.

The whole process is called: The whole process is called: hemophilic arthritis.hemophilic arthritis.

Haemophilic arthropathy (radiographs)Haemophilic arthropathy (radiographs)

Other types of bleeding: Other types of bleeding: subcutaneous, intramuscular hematomas, gastrointestinal subcutaneous, intramuscular hematomas, gastrointestinal bleeding, hematuria, cerebral hemorrhagebleeding, hematuria, cerebral hemorrhage

Volkmann’s contractureVolkmann’s contracture

Large haematoma of the Large haematoma of the cerebellum cerebellum (computer tomography)(computer tomography)

PseudotumorPseudotumor

Life-threatening bleeding:Life-threatening bleeding: - bleeding within the head is a - bleeding within the head is a major cause of death in major cause of death in haemophiliahaemophilia

-Bleeding into the throat may Bleeding into the throat may cause swelling, as well as difficulty cause swelling, as well as difficulty swallowing and breathingswallowing and breathing

- Gastrointestinal bleeding (often Gastrointestinal bleeding (often due to peptic ulceration)due to peptic ulceration)

Serious, but usually not Serious, but usually not life-threatening bleeding:life-threatening bleeding: - bleeds into the eyes, spine and - bleeds into the eyes, spine and

psoas musclepsoas muscle

TherapyTherapy

The only mode of treatment is The only mode of treatment is replacement therapy:replacement therapy: to to injectinject the missing clotting factor into a vein. the missing clotting factor into a vein. Clotting factor cannot be given by mouth.Clotting factor cannot be given by mouth.

Factor substitutionFactor substitution• On demand:On demand:

– in the event of bleeding in the event of bleeding episodesepisodes

• Profilaxis:Profilaxis: to to prevent bleedings and their prevent bleedings and their consequencesconsequences– primaryprimary– secundarysecundary

• Home treatment:Home treatment: – the patient or his relatives are the patient or his relatives are

taught to give iv. injection of taught to give iv. injection of the factor concentrate the factor concentrate immediately when there are immediately when there are symptoms of bleeding. symptoms of bleeding.

Calculation of the dose of factor replacementCalculation of the dose of factor replacement

Haemophilia A:Haemophilia A:(desired level FVIII % - patient FVIII level %) x bodyweightkg/2(desired level FVIII % - patient FVIII level %) x bodyweightkg/2

Haemophilia B:Haemophilia B:(desired level FIX % - patient FIX level %) x bodyweightkg(desired level FIX % - patient FIX level %) x bodyweightkg

Recommended doses of FVIII/FIX for various Recommended doses of FVIII/FIX for various types of haemorrhagetypes of haemorrhage

SSiittee ooff hheemmoorrrrhhaaggee:: DDeessiirreedd FFVVIIIIII//FFIIXX lleevveell ((%%))::

DDuurraattiioonn ((ddaayyss))::

Hemarthroses, superficial, intramuscular hematoma, im. inj.

1100--2200 %% 11--33

Deep intramuscular haematomas

2200--3355 %%

33--44

Tooth extraction, intraabdominal, intrathoracal bleeds, epistaxis, minor surgery

4400--5500 %%

44--1144

uunnttiill hheeaalliinngg

Central nervous system, major surgery

5500--110000 %%

1144--2211

uunnttiill hheeaalliinngg

Factor replacement Factor replacement at the consulting at the consulting

roomroom

Home therapy:Home therapy:

is infusion with clotting factor is infusion with clotting factor replacement away from the hospital. replacement away from the hospital. A person with haemophilia can infuse A person with haemophilia can infuse at home, school, work, or elsewhere.at home, school, work, or elsewhere.

sharps container sharps container disposable wipes disposable wipes alcohol wipe alcohol wipe bandage bandage cotton balls cotton balls tape tape tourniquet tourniquet butterfly needle butterfly needle syringe syringe transfer needle/transfer needle/filter needle filter needle factor concentrate factor concentrate latex gloves latex gloves diluent (sterile water) supplied diluent (sterile water) supplied with the concentrate with the concentrate

Supplies needed for treatment with factor concentrate: Supplies needed for treatment with factor concentrate:

A written record A written record of all treatments of all treatments must be kept. must be kept.

Medical treatment is only one part of good health.Medical treatment is only one part of good health.

People with hemophilia should:People with hemophilia should:

- Exercise and stay fit. - Exercise and stay fit. - Wear protection that is appropriate for the sport or - Wear protection that is appropriate for the sport or activity. activity. - Get regular check-ups that include joint and muscle - Get regular check-ups that include joint and muscle examination. examination. - Get all vaccinations recommended, including hepatitis A - Get all vaccinations recommended, including hepatitis A and hepatitis B protection. and hepatitis B protection. - Maintain a healthy body weight. People who do not exercise - Maintain a healthy body weight. People who do not exercise are more likely to put on extra weight. A person with are more likely to put on extra weight. A person with hemophilia needs to control his weight so that he does not hemophilia needs to control his weight so that he does not put extra stress on his joints, especially if he has arthritis. put extra stress on his joints, especially if he has arthritis.

Dental healthDental health is very important in haemophilia:is very important in haemophilia:

- Healthy teeth and gums reduce the need for haemophilia - Healthy teeth and gums reduce the need for haemophilia treatment. treatment.

- Regular dental care reduces the need for injections and - Regular dental care reduces the need for injections and surgery. surgery.

- Dental care should include brushing, flossing, and check-ups by Dental care should include brushing, flossing, and check-ups by a dentist. a dentist.

- Cooperation between hematologists (hemostaseologists) and Cooperation between hematologists (hemostaseologists) and dentists is necessary.dentists is necessary.

Regular dental visitsRegular dental visits – usually every 6 months – – usually every 6 months – will help identify problems early.will help identify problems early.

I. I. Prevention:Prevention:

It is an essential component of oral care. It is an essential component of oral care. It will reduce the need of treatment and the number It will reduce the need of treatment and the number of emergency visit.of emergency visit.

With prevention Without preventionWith prevention Without prevention

Oral hygiene is very important:Oral hygiene is very important:

BrushingBrushing twice daily with a fluoride toothpaste. twice daily with a fluoride toothpaste. Proper brushing is essential for cleaning teeth and gums Proper brushing is essential for cleaning teeth and gums effectively.effectively. 

It removes It removes plaqueplaque from the surfaces of teeth.from the surfaces of teeth.                                               

PlaquePlaque develops into unhealthydevelops into unhealthy calculus.calculus.

EnamelEnamel

PlaquePlaque

CalculusCalculus

Brushing must begin at Brushing must begin at childhood.childhood.

The toothbrush should have medium texture bristles The toothbrush should have medium texture bristles (- hard bristles can cause abrasion of the teeth(- hard bristles can cause abrasion of the teeth - soft bristles are inadequate).- soft bristles are inadequate). Interdental cleaning aids (floss, tape, interdental Interdental cleaning aids (floss, tape, interdental brushes) should be used to prevent the formation of brushes) should be used to prevent the formation of dental caries and periodontal disease.dental caries and periodontal disease.

Recommended Fluoride Supplementation:

AGEAGE

FLUORIDE ION LEVEL IN DRINKING WATER FLUORIDE ION LEVEL IN DRINKING WATER (ppm)

Less than 0.3 ppm

0.3 to 0.6 ppmMore than 0.6 ppm

Birth - 6 months NONE NONE NONE

6 months - 3 years 0.25 mg / day NONE NONE

3 - 6 years 0.50 mg / day 0.25 mg / day NONE

6 - 16 years 1.0 mg / day 0.50 mg / day NONE

Fluoride supplementsFluoride supplements (fluorid drops, tablets, topical application of fluorid (fluorid drops, tablets, topical application of fluorid mouthrinses)mouthrinses) are recommended if the water supply is are recommended if the water supply is 0,6 0,6 ppmppm

Dietary CounselingDietary Counseling

The consumption of food and The consumption of food and drinks with a high sugar and acid drinks with a high sugar and acid content should be limited to content should be limited to mealtimesmealtimes

(the aim: food and drink does not (the aim: food and drink does not cause the pH level of the oral cause the pH level of the oral cavity to fall below the critical cavity to fall below the critical level of pH 5,5).level of pH 5,5).

Artificial sweeteners (aspartame, Artificial sweeteners (aspartame, sorbitol, acesulfamate) can be sorbitol, acesulfamate) can be used as an alternative to sugars. used as an alternative to sugars.

II. II. Dental treatmentDental treatment

It is essential to prevent accidental damage to the It is essential to prevent accidental damage to the oral mucosa.oral mucosa.

Injury can be avoided by:Injury can be avoided by:

- careful use of saliva ejec- careful use of saliva ejectors

- careful removal of impressions

- care in the placement of X-ray filmscare in the placement of X-ray films

- protection of soft tissues during protection of soft tissues during restorative treatment.restorative treatment.

Orthodontic treatment:Orthodontic treatment:

Fixed and removable orthodontic appliences may be used. Fixed and removable orthodontic appliences may be used.

Special care should be taken when treating patients with a Special care should be taken when treating patients with a severe bleeding disorder to ensure that the gingiva is not severe bleeding disorder to ensure that the gingiva is not damaged when fitting the applience.damaged when fitting the applience.

Periodontal treatment:Periodontal treatment:

GUM DISEASE IS THE GUM DISEASE IS THE MAIN CAUSE OF TOOTH MAIN CAUSE OF TOOTH LOSS!LOSS!

Mouth tissues reflect Mouth tissues reflect symptoms of other problems.symptoms of other problems.80 % or more of adults have 80 % or more of adults have some form of periodontal some form of periodontal disease and disease and 99% of those have no signs 99% of those have no signs that indicate they have a that indicate they have a problem.problem.

Periodontitis (gum disease)Periodontitis (gum disease)Healthy gumsHealthy gums

Severely advanced periodontal Severely advanced periodontal (gum) disease, with receded (gum) disease, with receded gums, massive stains from gums, massive stains from smoking cigarettes and smoking cigarettes and inadequate oral hygiene. inadequate oral hygiene.

In case of periodontal disease treatment must start as soon as In case of periodontal disease treatment must start as soon as possible.possible.

Factor replacement therapy is not necessary.Factor replacement therapy is not necessary.

Bleeding can be controlled locally: with direct pressure Bleeding can be controlled locally: with direct pressure and topical antifibrinolytic agents.and topical antifibrinolytic agents.

Clorhexidine gluconate mouthwash can be used to Clorhexidine gluconate mouthwash can be used to control periodontal problems.control periodontal problems.

Antibiotics may be required to help reduce the initial Antibiotics may be required to help reduce the initial inflammation.inflammation.

Dental cariesDental caries

• If the oral hygiene is bad, If the oral hygiene is bad, certain bacteria have overgrown certain bacteria have overgrown on certain parts of the tooth on certain parts of the tooth surface and have produced so surface and have produced so much acid that the tooth much acid that the tooth mineral has dissolved or mineral has dissolved or decayed, forming a cavity. decayed, forming a cavity.

Carious lesionsCarious lesions

Restorative treatment:Restorative treatment:

Filling a cavityFilling a cavity can can be undertaken be undertaken routinely with routinely with protection the protection the mucosa.mucosa.

In the case of local In the case of local anesthesia factor anesthesia factor replacement replacement therapy is therapy is necessary.necessary.

Endodontic treatment (root canal therapy):Endodontic treatment (root canal therapy):

It is generally low risk for patients with bleeding disorders.It is generally low risk for patients with bleeding disorders.

Pulpectomy be carried out carefully (the instrument do not Pulpectomy be carried out carefully (the instrument do not pass through the apex of the root canal).pass through the apex of the root canal).

Tooth extraction, dental Tooth extraction, dental surgery:surgery:

Extraction of even a single tooth Extraction of even a single tooth requires replacement therapy requires replacement therapy (recommended FVIII/IX level: (recommended FVIII/IX level: 40-50 %).40-50 %).

Multiple extractions may save time Multiple extractions may save time and expense but create a major and expense but create a major bleeding hazard.bleeding hazard.

The suturing of bleeding tooth The suturing of bleeding tooth sockets should be avoided.sockets should be avoided.

Antifibrinolytics (tranexamic acid Antifibrinolytics (tranexamic acid = Exacyl) may diminish bleeding = Exacyl) may diminish bleeding in patients with coagulation in patients with coagulation disorders. disorders.

Anesthesia and pain management:Anesthesia and pain management:

- Minor analgesics:- Minor analgesics: dental pain can be controlled with a minor dental pain can be controlled with a minor analgesic, such as paracetamol. Aspirin should not be analgesic, such as paracetamol. Aspirin should not be used due to its inhibitory effect on platelet function!used due to its inhibitory effect on platelet function!

- Local anesthesia: - Local anesthesia:

No haemostatic cover:No haemostatic cover: Haemostatic cover required:Haemostatic cover required:

Buccal infiltrationBuccal infiltration Inferior dental blockInferior dental block

Intra-papillary injectionIntra-papillary injection Lingual infiltrationLingual infiltration

Intraligamentary injectionsIntraligamentary injections

Guidelines for Dental Treatment of Patients with Inherited Bleeding Disorders (WFH, Dental Committee, 2006)

Inferior alveolar nerve block:Inferior alveolar nerve block:

It is a risk of bleeding into the muscles along with It is a risk of bleeding into the muscles along with potential airway compromise due to a haematoma in potential airway compromise due to a haematoma in the retromolar or pterygoid space.the retromolar or pterygoid space.

A lingual infiltration:A lingual infiltration:

There is a risk of a significant airway obstruction in There is a risk of a significant airway obstruction in the event a bleed. the event a bleed.

Dental prosthesisDental prosthesis

Patients with bleeding disorders can be given Patients with bleeding disorders can be given dentures.dentures.

If a partial denture is provided it is important that the If a partial denture is provided it is important that the periodontal health of the remaining teeth is periodontal health of the remaining teeth is maintained.maintained.

The earliest known dental prosthesis The earliest known dental prosthesis from Rome from Rome (date from the 1st to the (date from the 1st to the 2nd century A.D.)2nd century A.D.) resulted from resulted from multi-karat gold wire, which was multi-karat gold wire, which was used to string together "artificial used to string together "artificial teeth." teeth."

They were found in the mouth of They were found in the mouth of an an unidentified woman unidentified woman who was buried who was buried in an elaborate mausoleum within a in an elaborate mausoleum within a Roman necropolis. Roman necropolis.

It is believed the unidentified It is believed the unidentified Roman's bridgework was made from Roman's bridgework was made from the woman's own teeth that probably the woman's own teeth that probably fell out due to periodontal disease. fell out due to periodontal disease. Gold wire bound the teeth together.Gold wire bound the teeth together.

In dental health of a patient with bleeding In dental health of a patient with bleeding disorder team work is very important disorder team work is very important

(patient- dentist- haematologist)(patient- dentist- haematologist)