Inherited platelet disorders and oral health

An early view of the above article has been published online in the Journal of Oral Pathology and Medicine. The article highlights the importance of platelets in haemostasis and their involvement in the formation of a platelet plug which halts further blood loss. However when a person suffers from a platelet disorder this process is incomplete and continuation of bleeding occurs. A full list of inherited platelet disorders can be found here.

This particular review looks at the ‘characterisation, clinical manifestations, therapy and dental management of the most common inherited platelet disorders’. Valera et al aimed to produce ‘possible protocols for the management of patients with platelet disorders requiring oral health care’.

The following table briefly outlines the most common inherited platelet disorders along with the oral complications that may arise:

Disorder

Defect

Complication

Oral manifestations

Bernard­-Soulier syndrome

reduced platelet attachment to von Willebrands Factor

during the initial steps of platelet adhesion

Severe bleeding

Gingival bleeding

Glanzmann thrombasthenia

quantitative or qualitative defects of the glycoprotein IIb ⁄IIIa

Moderate to severe bleeding

Gingival bleeding enhanced by poor OH,

Excessive bleeding following trauma

Platelet-type von Willebrand’s disease

spontaneous binding of plasma VWF to platelets

Prolonged bleeding time

Bleeding and haemorrhage following tooth extraction

Hermansky-­Pudlak syndrome

platelet dense granule deficiency

severe immunologic deficienc

increased vulnerability to severe gingivitis and periodontitis

Premature loss of teeth

Chediak-­Higashi syndrome (CHS)

platelet dense granule deficiency

severe immunologic deficiency

increased vulnerability to severe gingivitis and periodontitis

Premature loss of teeth

Gray platelet syndrome

defective retention of αgranule protein

mild to moderate mucocutaneous bleeding and thrombocytopenia

 

Paris-Trousseau  (Jacobsen syndrome)

deletion of the long arm of chromosome 11

mild haemorrhagic tendency

 

Quebec platelet syndrome

degradation of several a-granule proteins such as factor V, VWF, thrombospondin, and P-selectin

accelerated lysis of platelet­fibrin clots

 

Scott syndrome

reduced ability to convert prothrombin into thrombin

Bleeding tendency

 

Wiskott­-Aldrich syndrome

Impaired T lymphocytes and platelets

Susceptible to bacterial, viral and fungal infections

gingivalbleeding, oral petechiae, ulceration, and premature tooth loss

 

When patients suffering from an inherited platelet disorder require treatment anti-haemorrhagic agents may be used. These included Desmopressin, Antifibrinolytic agents, Transfusion and Recombinant activated factor VII.

Desmopressin

This is a ‘synthetic analogue of antidiuretic hormone vasopressin’. Its main functions are to ‘increase the plasma levels of factor VIII and VWF and platelet adhesiveness’. It can be administed by IV subcutaneously or by nasal spray.

Antifibrinolytic agents

These include Tranexamic Acid and ‘limit the endogenous lysis of fibrin clots by blocking the binding of plasminogen to fibrin and its subsequent activation to plasmin’. This agent is usually used to prevent and control minor mucosal bleeding, possibly after dental extractions. Tranexamic Acid is of oral administration or as a mouthwash.

Transfusion

Transfusions of platelets are used for those suffering from severe platelet function defects. They are used in cases of severe bleeding.

Recombinant activated factor VII

This is usually used got haemophilia patients with uncontrollable bleeding and a number of other congenital and acquired abnormalities. It is given to patients over a number of hours intravenously.

The authors summarise that ‘for mild bleeding, mouthwash with Tranexamic Acid, topical thrombin, fibrin glue, Desmopressin and antifibrinolytic drugs are the most relevant therapeutic approaches’. If there is severe bleeding then a platelet transfusion would be the first choice.

The authors then propose the following management options for dental treatments:

Oral health

Previous studies have suggested that caries and periodontal disease incidence is higher in patients with bleeding disorders. This has been felt to be related to the fear of bleeding when performing oral hygiene measures such as flossing and during professional dental treatments. With this in mind they suggest that patients with disorders should be given advice about dental hygiene and toothbrushing techniques. Depending on the patient it might be necessary to include advice on fluoride and the diet. It is also recommended that these patients receive regular dental check-ups, in order to review their oral hygiene status and provide gentle but thorough cleaning of the mouth preventing disease. For patients with CHS, antibiotic prophylaxis should be given prior to non-surgical and surgical dental procedures in the form of Amoxicillin 1000mg twice a day for seven days.

Anaesthesia

Inferior dental nerve blocks should be avoided in patients with bleeding disorders wherever possible. This is due to the risk of haemorrhage or haematoma formation and causing obstruction to the airway. If treatment is indicated appropriate therapy needs to be considered and arranged by the haematological department. They also suggest that an alternative technique be used along with an aspirating syringe and a local anaesthetic with a vasoconstrictor to reduce bleeding tendency. They propose that for an Inferior dental nerve block to be administered the platelet levels need to be raised above 30 G/l.

Oral Surgery

For certain surgical procedures a haematologist may be required to ‘confirm an adequate platelet response and adjust treatment as necessary’.

Non-invasive procedures and paediatric dentistry

Non-invasive procedures include restorations that do not involve the gingiva, crowns and bridges. However special care is required when adjusting prosthesis, placing clamps, matrices and wedges for restorations.

When extracting deciduous teeth the local haemostatic techniques outlined above should be sufficient. However in cases of trauma treatment options should be discussed with a paediatrician. Preventive methods such as fissure sealants and fluoride application would be appropriate for these patients. As with all patients, after anaesthesia, they should be warned regarding the risk of postoperative self-trauma. They state that ‘inadvertent trauma and periodontal bleeding secondary to stainless steel crown placement’ should be managed with local haemostatic agents. Care should also ‘be taken to avoid injuring the gingiva with orthodontic appliances’.

Post-operative bleeding

Prior to treatment, the risks of post-operative bleeding should be assessed and a decision made about the location for treatment, i.e. hospital. If post-operative bleeding occurred, reapplication of pressure packs and packing or repacking sockets should take place. Patients should be given post-operative advice, both written and verbal. If treatment was performed in surgery a phone call follow-up is suggested. For those treated in hospital ‘clinical surveillance during 24-48 hours is necessary.

The authors give a brief summary which includes:

·         Patients with mild inherited platelet disorders can be treated at the dental surgery for non-surgical procedures;

·         Surgical procedures and inferior dental nerve blocks need to be carried out in an oral surgery unit with systemic therapy;

·         Desmopressin and antifibrinolytic agents are the methods of choice;

·         Many patients can be treated with local haemostatic agents and pressure sutures

·         Patients with severe platelet disorders should be managed in conjunction with haematologists in a hospital environment.

In conclusion, patients suffering from inherited platelet disorders should be given preventive treatment routinely in order to prevent disease or further progression which in turn prevents the risk of bleeding and the need for transfusions and medications.

 

References:

Valera M.-C., Kemoun P., Cousty S., Sie P. and Payrastre B. (2012), Inherited platelet disorders and oral health. Journal of Oral Pathology & Medicine. doi: 10.1111/j.1600-0714.2012.01151.x http://onlinelibrary.wiley.com/doi/10.1111/j.1600-0714.2012.01151.x/abstract

Handin R I. Inherited Platelet Disorders. Hematology 2005 2005:396-402; doi:10.1182/asheducation-2005.1.396 http://asheducationbook.hematologylibrary.org/content/2005/1/396/T1.expansion.html

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