Blood Dyscrasias

Blood Dyscrasias
11.1 Thalassaemia

Section I: Clinical Scenario and Dental Considerations

Clinical Scenario

A 12‐year‐old girl presents to the dental clinic accompanied by her mother. The patient does not like the appearance of her teeth and feels that the shape of her face is changing.

Medical History

  • Beta‐thalassaemia major
  • Splenomegaly


  • Regular blood transfusion commenced 4 years ago when she moved countries due to her refugee status; prior to this only received transfusions intermittently
  • Vitamin B supplementation
  • Deferiprone – iron chelating agent

Dental History

  • Irregular dental attender; attends only when in pain
  • Stopped going due to problems with access and previous traumatic experience
  • Often feels tired and forgets to brush her teeth

Social History

  • Refugee – originally from Afghanistan; able to communicate in English
  • Lives with her mother who does not speak English and relies on her daughter to translate
  • Remaining family is in Afghanistan
  • Attends school
  • Diet includes high quantities of chocolate, biscuits and fizzy drinks

Oral Examination

  • ‘Chipmunk face’ (prominent malar eminence), saddle nose, frontal bossing and anterior open bite (Figure 11.1.1)
  • Lips – angular cheilitis
  • Soft tissue – pale mucosa
  • Gingivae – pale, plaque at cervical margins of teeth
  • Hard tissue – maxillary bone expansion/protrusion
  • Dentition – class II malocclusion (crowding), mixed dentition with mobility and caries in deciduous teeth (#53, #55, #63, #74, #75 and #84) (Figure 11.1.2)
  • Partially erupted teeth #33 and #34

Radiological Examination

  • Orthopantomogram undertaken (Figure 11.1.3)
  • All teeth of the permanent dentition are present and developing
  • Thin mandibular cortex
  • Maxillary sinus pneumatisation

Structured Learning

  1. How may the presence of a splenomegaly affect how you manage this patient?
    • Progressive splenomegaly may be associated with significant debilitating symptoms including:
      • Early satiety due to pressure on the stomach
      • Abdominal pain, predominantly in the left upper abdomen that may spread to the left shoulder
      • Inactivity and fatigue
      • Portal hypertension
      • Progression of cytopenias due to splenic sequestration may result in more frequent infections, anaemia and increased bleeding
    • In view of these factors, consider:
      • Avoiding a fully supine position if there is abdominal pain
      • Limiting the appointment length due to fatigue
      • Checking the results of a recent full blood count test due to potential cytopenias
    Photo depicts lateral view of the face showing malar prominence and anterior open bite (S).

    Figure 11.1.1 Lateral view of the face showing malar prominence and anterior open bite.

    Photo depicts mixed dentition with generalised crowding and pale gingivae (S).

    Figure 11.1.2 Mixed dentition with generalised crowding and pale gingivae.

    Photo depicts orthopantomogram demonstrating mixed dentition, thin mandibular cortex and maxillary sinus pneumatisation (M).

    Figure 11.1.3 Orthopantomogram demonstrating mixed dentition, thin mandibular cortex and maxillary sinus pneumatisation.

  2. What would you need to consider in relation to the regular blood transfusions?
    • Avoid invasive dental care on the same day as the transfusion
      • Transfusions make take up to 4 hours to administer and the patient is often tired
      • The patient may experience side‐effects from transfusion, including fever, allergy and more acute reactions such as haemolysis
    • Dental treatment should be planned for the week following a blood transfusion as this will ensure that the patient’s blood counts are optimal
    • Determine the presence and extent of transfusion‐related iron overload complications and implement precautions as required
  3. What factors could be contributing to the patient’s perception that her teeth and face are changing in appearance?
    • Thalassaemia‐associated changes in the orofacial tissues may be observed in more severe forms of beta‐thalassaemia – this patient has the most severe form, namely ‘major’
    • These changes are also more likely to present when regular blood transfusions are not given at an early age, as is the case for this patient
    • Many of these are related to expansion of the marrow cavity due to compensatory hyperplasia of bone marrow (extramedullary haematopoiesis)
    • The hyperplasia of bone marrow in the maxilla exceeds that of the mandible, and results in a characteristic appearance known as ‘chipmunk face’ (Figures 11.1.1 and 11.1.4); this may be associated with spacing of the upper teeth, forward drift of the maxillary incisors and increased overjet
      Photos depict (a,b) Facial features: frontal bossing, ‘chipmunk face’, class II malocclusion (S).

      Figure 11.1.4 (a,b) Facial features: frontal bossing, ‘chipmunk face’, class II malocclusion.

    • The patient is also in the mixed dentition stage and undergoing puberty which may also impact on the change in her facial and dental profile
  4. What risk factors does this patient have for the development of angular cheilitis?
    • Angular cheilitis is an inflammation at the corners of the mouth, typically caused by infection (bacterial and/or fungal), irritation and/or allergies
    • Risk factors for this patient include:
      • Underlying anaemia due to thalassaemia/splenomegaly
      • Increased risk of infection related to cytopenia caused by splenomegaly
      • Anterior open bite and pronounced Class II skeletal base, resulting in saliva leakage in the corners of the mouth
  5. When you discuss the patient’s poor dental health with the patient and her mother, they both state that the teeth have deteriorated as they are ‘weak’ due to the thalassaemia. Are they correct?
    • Tooth deformities (taurodontism) and spacing of teeth may occur in relation to thalassaemia; however, the tooth structure itself is not at more risk of developing dental caries
    • It has been noted that the saliva in patients with thalassaemia contains less phosphorus and IgA, and hence may be less protective
    • Increased dental caries risk is more likely to be due to compliance issues, poor dental attendance, poor oral hygiene and highly cariogenic diet, tendency to eat small amounts of food but frequently (due to splenomegaly)
  6. What factors do you need to consider in your risk assessment when planning dental care for this patient?
    • Social
      • Dependent on parents to attend
      • Mother unable to speak English – translator required as it is not appropriate for her daughter to translate for her
      • Past traumatic dental experience
      • Poor self‐esteem due to concerns about her facial appearance
      • Potential economic constraints (refugee status)
    • Medical
      • Regular blood transfusions
      • Anaemia
      • Increased risk of bleeding due to hypervascularity of bone (extramedullary haematopoiesis)
      • Risk of blood‐borne virus spread
      • Risk of infection (sequestration of cells due to splenomegaly)
    • Dental
      • Past traumatic dental experience
      • Mixed dentition and crowding, may need orthodontic consultation
      • Angular cheilitis – care should be taken not to irritate the fissures
      • Highly cariogenic diet
      • Poor dental attendance
  7. The patient asks if there is an operation that she can have to improve her facial appearance by making her top jaw smaller. What would you advise her?
    • Surgical correction of the facial profile may not be stable, particularly as the patient is still growing
    • There is also a significant risk associated with surgical approaches and the maxillary bone in particular is hypervascular (risk of extensive bleeding)
    • The focus should be on preventive approaches and stabilisation of oral health
    • Orthodontic treatment can be initiated as early as possible once this has been achieved
    • Correction of drifted maxillary anterior teeth and increased overjet should be undertaken to improve aesthetics, reduce susceptibility to trauma, avoid gingival inflammation and improve functional ability

General Dental Considerations

Oral Findings

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Nov 6, 2022 | Posted by in Implantology | Comments Off on Blood Dyscrasias

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