Section I: Clinical Scenario and Dental Considerations

Clinical Scenario

A 29‐year‐old female diagnosed with type 1b glycogenosis presents to the dental clinic complaining of bleeding gums and mobility of several lower teeth. She reports that her symptoms have worsened in recent weeks.

Medical History

  • Type 1b glycogenosis (von Gierke disease), a rare inherited condition caused by mutations in the SLC37A4 gene, characterised by glycogen and fat build‐up within the liver and kidneys, that causes:
    • Hypoglycaemic episodes
    • Neutropenia
    • Myeloid cell line dysfunction
    • Recurrent mouth ulcers
    • Hepatomegaly
    • Inflammatory bowel disease
    • Microalbuminuria
  • Recurrent epistaxis
  • History of hip abscess (8 years earlier)
  • History of appendectomy (22 years earlier)


  • Filgrastim (r‐metHuG‐CSF)
  • Mesalazine
  • Vitamin D3
  • Enalapril

Dental History

  • Regular dental attender in your practice; also visits the dental hygienist every 12 weeks
  • Meticulous oral hygiene habits; uses a soft toothbrush and does not use interdental brushes as advised by her physician
  • Brushes teeth 3 times a day and uses a 0.12% chlorhexidine mouthwash
  • History of recurrent, painful and incapacitating mouth ulcers
  • Pericoronitis of the mandibular third molar (1 episode)

Social History

  • University educated but currently unemployed
  • Lives at home with parents
  • Attends appointments accompanied by her father, who is a physician by profession

Oral Examination

  • Good oral hygiene
  • Significant gingivitis
  • Generalised bleeding on probing
  • Generalised tooth mobility, with grade II mobility of the lower anterior teeth
  • Fillings in #15, #25, #35 and #37

Radiological Examination

  • Orthopantomogram undertaken (Figure 11.3.1)
  • Multiple dental diastemas
  • Tooth root resorption of the mandibular incisors
  • Impacted tooth #38

Structured Learning

  1. Why is this patient on filgrastim (r‐metHuG‐CSF)?
    • Filgrastim is a recombinant human granulocyte colony stimulating factor (G‐CSF) therapy
    • The recombinant protein resembles the natural factor, releasing the neutrophil reservoirs from the bone marrow to the peripheral bloodstream, increasing production and acting as an antiapoptotic factor
    • This can lead to a 10–12‐fold increase in the neutrophil count and result in a higher life expectancy for this patient
      Photo depicts panoramic radiography showing tooth resorption of the mandibular incisors and impacted tooth number 38 (M).

      Figure 11.3.1 Panoramic radiography showing tooth resorption of the mandibular incisors and impacted tooth #38.

  2. What adverse effects can filgrastim (r‐metHuG‐CSF) have in the oral cavity?
    • Bleeding gums due to thrombocytopenia
    • Ulcers
    • Swelling (lips and/or tongue)
    • Mucositis
    • Cracked lips
  3. The patient is very distressed that her lower teeth are mobile. How would you explain to her why this has happened, despite the fact that she cleans her teeth 3 times a day and visits the dental clinic regularly?
    • Although G‐CSF can improve the control of bacterial infections in individuals with neutropenia, many patients still experience these infections such as pneumonia, other respiratory infections, stomatitis and severe persistent gingival inflammation
    • Periodontal manifestations may range from marginal gingivitis to rapidly progressive periodontal disease with advancing bone loss, which may affect both primary and permanent dentition, but primarily the latter
    • Despite her regular toothbrushing, the use of a soft toothbrush, coupled with a lack of interdental cleaning and increased susceptibility to aggressive periodontal disease due to neutropenia, may result in rapid bone loss
  4. In the first instance, you recommend a course of periodontal treatment including scaling and root planing. What factors are considered important in assessing the risk of managing this patient?
    • Social
      • The patient has a rare inherited condition and her father is a physician; both are likely to question the knowledge of this condition by the dentist and the awareness of the risks and how to mitigate against these
    • Medical
      • Increased risk of local and focal infection due to neutropenia
      • Risk of hypoglycaemia during and mainly following dental treatment sessions
      • Bleeding tendency based on recurrent epistaxis, potential platelet dysfunction (myeloid cell line dysfunction) and filgrastim administration (decreases platelet count)
    • Dental
      • The patient requires intensive periodontal treatment
      • Mandibular incisors have poor prognosis
      • Assess extraction of tooth #38
  5. In order to fully assess the risk of undertaking root planning, preoperative blood tests are advisable. What tests should you request?
    • Full blood count
      • Haemoglobin and haematocrit (risk of anaemia)
      • Red blood cell count (risk of anaemia)
      • Neutrophil count (risk of infection)
      • Platelet count (risk of bleeding)
      • Coagulation study (risk of bleeding due to hepatic dysfunction)
    • Blood biochemistry
      • Blood glucose (risk of hypoglycemia)
      • AST, ALT and GGT (to assess hepatic function)
      • Albumin and creatinine (to assess renal function)
  6. The laboratory tests reveal a neutrophil count of 1 × 109/L. After consulting with the patient’s physician, they recommend performing the dental treatment in a hospital setting. Why?
    • Myeloid dysfunction is common in type 1b glycogenosis; therefore, a sufficient blood cell count (e.g. neutrophils) does not imply that these are functionally adequate and therefore does not ensure that complications will not arise
    • Dental treatment can affect oral intake in the subsequent hours/days and, as a result, promote the onset of hypoglycaemic episodes (a complication that can be severe for this patient)
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Nov 6, 2022 | Posted by in Implantology | Comments Off on Neutropenia

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