Dental and Oral Complications of Bleeding Disorders

• Bleeding disorders generally involve problems with one or more of the following:

  • Vascular (vessel wall) component
  • Platelets (the first cells to react to a break in the vessel wall)
  • Coagulation factors
  • Fibrin
• Vascular problems can be:

  • Congenital - eg Ehlers Danlos (a problem with collagen - resulting in hypermobile joints and capillary fragility, so can bruise easily)
  • Acquired - eg scurvy (vitamin C deficiency - again affects collagen resulting in easy bruising)
  • May be less of an issue clinically e.g. Most cases of Ehlers Danlos - because just concern capillary fragility, so would stop bleeding after extraction same as anyone else
• Platelet disorders are either:

  • Disorder of function - congenital or acquired - may have high number of platelets, but they might not be working properly

  OR

  • Change in numbers through: increased/decreased production/destruction
• Functional platelet disorders - examples:

  • Congenital: von Willebrand's disease
  • Acquired functional disorders such as Aspirin induced, or Renal function induced
• Functional platelet disorders - examples:

  • Congenital: von Willebrand's disease
  • Most common coagulopathy 1/10,000
  • Spectrum  of mild-severe (type I to type IV) dependent on amount of vWF present
  • von Willebrand's disease = low levels of function of vWF released from endothelial cells and platelets
  • vWF plays an important role in helping the platelets to stick together and carries Factor 8 in the circulation and helps deliver it to the site of injury
  • Can use DDAVP in mild vWd to help reverse it long enough to treat the pt - in more severe forms, may need Factor 8 replacement before treatment
• Functional platelet disorders - examples:

  • Acquired functional disorders such as Aspirin induced, or Renal function induced
  • Aspirin creates problems with platelets due to its inhibition of the cyclooxygenase system - analgesia lecture
  • Induced renal function inhibits the aggregation of platelets - they can't stick to the wound or together to form a clot - caused by glycoprotein IIb-IIIa deficit
• Platelet disorders - change in numbers:

  • Thrombocytopenia (low levels of platelets - less than 150x10^9)
  • Thrombocytosis (high levels of platelets >400x10^9)
• Platelets are formed in bone marrow from megakaryocytes, which are then progenitor cells. Anything that causes damage to the bone marrow (eg chemotherapy) will cause decreased formation of platelets.
• Platelet disorders - change in numbers:

  • Thrombocytopenia (low levels of platelets - less than 150x10^9)
  • Most common
  • Results are from either:
  • Decreased formation = abnormal bone marrow
  • OR
  • Increased destruction = normal bone marrow - but may be an autoimmune process (eg immune thrombocytopenic purpura) that means they destruct quicker - could be due to splenomegaly (enlargement of spleen) or disseminated intravascular coagulation
  • Causes: alcoholic liver disease, or malignancy
• Platelet disorders - change in numbers:

  • Thrombocytosis (high levels of platelets >400x10^9)
  • Just because there are too many doesn't immediately mean they all work well...clot vs bleed
  • Too many and all working well -> more clotting, so more at risk of DVT (deep vein thrombosis) or a pulmonary embolism
  • Too many and don't work v well -> risk of bleeding
• Platelet disorders - change in numbers:

  • Thrombocytosis (high levels of platelets >400x10^9)
  • Results from one of the following:
  • Reactive or secondary thrombocytosis - reacted to another disease process eg chronic inflammation
  • Familial thrombocytosis - genetic
  • Clonal thrombocytosis, including essential thrombocythemia and related myeloproliferative disorders (like multiple myeloma - a bone-based neoplastic process)
• Coagulation disorders are either:

  • Intrinsic defects - affect intrinsic pathway
  • Indicated by prolonged APTT (Activated Partial Thromboplastin Time)
  • Can be congenital eg Haemophilia A & B, or acquired e.g. Use of Heparin (used to thin the blood)
  • Extrinsic defects - affect extrinsic pathway (more interested in this one)
  • Prolong PT (prothrombin time) - generally tend to be acquired e.g. Liver disease, warfarin etc.
• Haemophilia A = factor VIII (8) deficiency
• Haemophilia B = factor IX (9) deficiency
• Fibrin disorders are either the result of:

  • Thrombolytic therapy e.g. Streptokinase
  • Used in MI (myocardial infarction) cases to break down blood clots
  • Pathological fibrinolysis e.g. tumour (makes plasmin in plasma digest fibrin)
• Medical history by system (key things to look for):

  • GIT/liver
  • Liver disease eg primary billiary cirrhosis or alcoholic-related liver disease
  • Chronic renal failure - impaired platelet function - defective aggregation of platelets; decreased glycoprotein IIb - IIIa
• Medical history by system (key things to look for):

  • Ehlers Danlos
  • Thrombophilias - Factor V Leiden deficiency, Antithrombin III deficiency, Antiphospholipid syndrome (Lupus anticoagulant) - cause an increased likelihood to clot - therefore need to take anticoagulants
• Medical history by system (key things to look for):

  • Coagulation system
  • Haemophilias
  • Disorders of bone marrow
  • Leukaemias (ALL)
  • Myelodysplasia
  • Platelet disorders
  • Thrombocytopenia
  • von Willebrand's Disease
  • Evidence (and or probing on) bleeding problems
  • Epistaxis, bruising, family history
  • Previous haemostatic challenges: surgery
• Medical history - medications:

  • Anti-platelets - aspirin, clopidogrel, prasugrel
  • Anti-coagulants - warfarin, heparin (LMWH - low molecular weight heparin - tinzaparin, enoxoparin, new Direct Oral Anticoagulants (DOAC), dabigatran (works on Factor IIa) - Rivaroxaban, Apixaban & Edoxaban, (all work on Factor Xa)
  • Myelosuppressive (suppress the bone marrow) - DMARDs and chemotherapy
• DMARDs are disease modifying antirheumatic drugs - eg methotrexate - cause bone marrow suppression due to their action on DNA synthesis - therefore suppress the function of megakaryocytes and other cells - therefore need to make sure platelet levels and white cell counts are normal
• Social history - alcohol:

  • How many units/week
  • How long for
  • If it's  >40 units a week for a long time - how might their liver be functioning
  • Liver has 2 functions: creates clotting factors 2, 7, 9 & 10 as well as albumin, and the other function is metabolic: it breaks down drugs
  • If synthetic function is not working then clotting times will change
  • Should take full blood count - platelet count should be 150-400x0^9 - PT should be 13-15s - APTT will be measured too, but isn't important for what we do as dentists
• Social history - dental:

  • Previous extractions - possible haemostatic challenges
  • Bleeding gingiva - if spontaneous/profuse then could indicate bleeding tendency
  • Previous problems and management required
• What to look for when examining a pt with a bleeding disorder:

  • Bleeding into skin
  • Vascular abnormalities
  • Other visual clues of underlying disease
• What to look for when examining a pt with a bleeding disorder - bleeding into skin:

  • Ecchymoses (bruising) vs purpura
  • Ecchymoses
  • Bleeding into subcutaneous tissues ("bruising") usually due to trauma
  • Will vary in appearance dependent on skin tone and colour
  • Key question to ask (yourself): is trauma appropriate to extent of bruising
• What to look for when examining a pt with a bleeding disorder - bleeding into skin:

  • Ecchymoses (bruising) vs purpura
  • Purpura
  • "Blood spots"
  • Caused by spontaneous bleeding into skin causing pools of blood within the skin
  • Can be either:
  • Non-thrombocytopenic purpura (not due to low levels of platelets)
  • OR
  • Thrombocytopenic purpura (TP) (due to low levels of platelets)
• Non-thrombocytopenic purpura (not due to low levels of platelets) - causes include:

  • Hereditary haemorrhagic telangiectasia
  • Ehlers Danlos
  • Infections
  • Deficiencies
  • Allergy or connective tissue diseases
  • Ageing skin "senile purpura" or actinic purpura - due to reduction in connective tissue, loss of subcutaneous fat to support the overall skin structure, flattening of the dermal junction (epidermis doesn't adhere as well to the dermis) or effacement of the dermal papillae

  Sign rather than a diagnosis of bleeding disorder - therefore need to do thorough investigation through history
• Thrombocytopenic purpura (TP) (due to low levels of platelets):

  • Usually comes with petechiae (small blood spots in the skin):
  • 1-3mm in diameter
  • Not disappear on pressure
  • Disappear  after 3-4 days
  • Signify abnormal capillary fragility, thrombocytopenia, or coagulopathy
  • May appear on hard palate too
• Thrombocytopenic purpura (TP) (due to low levels of platelets) - causes:

  • Impaired production of platelets
  • Generalised bone marrow failure - leukaemias, aplastic anaemias, myeloma
  • Selective  reduction in megakaryocytes (produce platelets) due to drugs eg Co-Trimoxazole or alcohol
  • Excessive platelet destruction
  • Immune problems - autoimmune/triggered by particular viral infections - could also be triggered by large blood transfusions due to trauma
  • Coagulation problems
  • Sequestration of platelets in spleen
  • Dilutional loss following massive transfusions
• What to look for when examining a pt with a bleeding disorder - vascular abnormalities:

  • Telangiectasia
  • Haemangioma
• Telangiectasia = dilation of small veins:

  • Normally benign but can signify underlying disease
  • 2 important ones to be aware of:
  • Spider telangiectasia
  • Radiate from central spot
  • Disappear on pressure, refill
  • Common in hepatic cirrhosis - but doesn't mean you have to have it if you have spider telangiectasia
  • Hereditary Haemorrhagic Telangiectasia
  • Dilated vessels up to 2mm in diameter
  • Face, mucous membranes of mouth and nose
  • Epistaxis (nosebleeds) is common and GIT bleeds may lead to anaemia
  • Clinical problem if traumatised - but generally not issue for dentists (despite I/O presentation)
• Haemangioma:

  • Abnormality of blood vessels
  • Blanch on pressure
  • Compressible & unsure - ultrasound or FNA (fine needle aspirate)
  • Essentially small benign mesenchymal tumours formed by blood vessels that display a rapid increase in cell proliferation - grow rapidly and regress slowly and don't generally reoccur
  • If small and mucosal away from teeth can be insignificant unless traumatised
  • If involving alveolar mucosa and extensive consider possible bony involvement - if that's the case then don't start doing things to traumatise the teeth eg extractions/surgery - get radiographs first
• Other visual clues of underlying disease in a pt with a bleeding disorder:

  • Alcohol abuse
  • Liver disease
  • Ecchymosis inappropriate to level of trauma
  • Jaundice
  • Sialosis
  • Ascites (excessive accumulation of fluid in the abdominal cavity due to imbalance in oncotic pressure) & fluid retention
  • Spider telangiectasia (aka spider naevi)
• Liver disease:

  • Affects metabolic &/or synthetic function
  • One of synthetic functions hepatocytes = Vitamin K dependent clotting factors (II, VII, IX, X)
  • Without these can see difficulties in the extrinsic pathway
  • Portal venous hypertension
  • Causes splenomegaly & sequestration of platelets; keeps platelets in instead of letting them out -> thrombocytopenia
• Liver disease:

  • Obstruction of bile duct
  • Cause decreased fat digestion => steatohorrea (fatty stools that float + smell bad)
  • Steatohorrea also => decreased absorption of Vitamin K; it's fat soluble => additive effect on inability to synthesise II, VII, IX & X
• Blood tests in pts with bleeding disorders - APTT (Activated Partial Thromboplastin Time):

  • Tests intrinsic pathway of clotting cascade
  • Affected by most factor deficiencies
• Blood tests in pts with bleeding disorders - PT (Prothrombin Time):

  • Tests extrinsic and common pathway of clotting cascade
  • Affected by warfarin and alcoholic liver disease
• Blood tests in pts with bleeding disorders - FBC: platelet count (150-400x10^9) as well as red cell and white cell count.
• Blood tests in pts with bleeding disorders - liver function tests - generally don't need these if just working under LA:

  • Test synthesis and excretion
  • Tests show decreased synthesis: decreased albumin, decreased coagulation factors (increased PT)
  • Tests show decreased excretion (cholestasis - bilirubin isn't getting where it needs to): increased bilirubin, alkaline phosphatase, GGT (gamma glutamyl transferase)
  • Tests show hepatocyte necrosis: increased AST & ALT (their increase is due to liver cell damage)
• Risk procedures:

  • Extractions
  • Perio surgery
  • Matrix bands
  • Suction
  • Block anaesthesia
• Pre-emptive management of bleeding:

  • Bleeding disorder => liaise appropriately
  • Haematologist - if it's a congenital bleeding disorder like vWD or haemophilia
  • Oncologist - if it's an acquired bleeding disorder like thrombocytopenia due to chemotherapy
  • Hepatologist if it's acquired through liver disease
  • Number of local measures to manage bleeding generally - use these in conjunction with other management techniques from suggestions on liaison