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was generalized mild to moderate gingival  Ocular Modifications            required for blood clotting and are specifi-
      inflammation.  Probing  depths  and  clinical  HPS  patients  experience  ocular complica-  cally involved in the secondary aggregation
      attachment level ranged from 2-5 mm with  tions and sensitivity, such as nystagmus,  response.   HPS patients can present with
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      generalized  bleeding  upon probing in all  photophobia,  and general  reduction  in vi-  an absence  of delta  granules  resulting  in
      four quadrants, as well as class I mobility  sual acuity.  Previous literature has recom-  impaired blood clotting. This complication
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      on teeth #8 and 9. In addition, teeth #11 and  mended that HPS patients should be given  can especially manifest several hours after
      25 showed Miller class I recession on the  UV-filtering  eyewear  to  accommodate  po-  injury or surgical procedure, as the formed
      facial surfaces.  Detectable plaque was seen  tential  light sensitivity and similar ocular  clot is unstable. The only way to confirm the
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      particularly  on the  proximal  and  lingual  complications  that  may occur under the  status of delta granules is by examining pre-
      aspects of molars and mandibular incisors.  bright lighting of the dental clinic.  In cases  pared platelets under electron microscope.
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      Calculus  deposits were clinically  detected  that demonstrate a more severe presentation  Patient platelet numbers are typically nor-
      on multiple teeth and were confirmed by the  of HPS, these modifications should be im-  mal, but their function is impaired.  While
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      radiographic exam (Figure 5). The patient  plemented.                      a bleeding time test could be beneficial, this
                                                                                 test is insensitive and lacks reproducibility,
                                            Gastrointestinal Modifications       therefore it is not recommended.  Instead,
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                                            It is common for patients with HPS to also  providers can request the newer and more
                                            experience  inflammatory  bowel  disease  accurate  platelet  function  analyzer  (PFA-
                                            (IBD) or ulcerative colitis (UC). 11-13  Because  100) test to determine  the patient’s clot-
                                            IBD may precipitate  ulcers and lesions in  ting ability.  Prothrombin time and partial
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                                            the  oral  cavity, a  thorough  intraoral  exam  thromboplastin time tests would be normal
                                            by the clinician  is important  to recognize  and a complete blood count would not pro-
                                            these lesions. 13, 14   Therefore,  the provider  vide any information on blood clotting sta-
                                            must recognize that a patient with HPS pre-  tus, as HPS affects the quality of platelets,
                                            senting with these lesions in the oral cavi-  not the quantity. Currently, there are no clin-
         Figure 5. Bitewing radiographs.
                                            ty may also have IBD and should warrant  ical practice guidelines for management of
      was diagnosed  with  class  I malocclusion  further investigation if a diagnosis of IBD  bleeding in HPS. Based on the severity of
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      and generalized mild periodontitis. Further,  has not been made.  The presence of IBD  the symptoms, the patient should be evalu-
      he displayed anterior wear loss without loss  or UC can affect a patient’s oral health, as  ated by a hematologist. In addition, aspirin
      of vertical dimension of occlusion due to  previous literature has shown a correlation  should be avoided in these patients due to its
      supraeruption  of the  mandibular  anterior  between the presence of UC, increased rates  impact on blood clotting. 4
      teeth, corresponding to Turner classification  of periodontal diseases, and higher decayed,
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      category 2. 9                         missing,  or  filled  teeth  (DMFT)  scores.   When a patient presents with a bleeding dis-
                                            Additionally, medication used to treat IBD  order, the provider should obtain as much
      Treatment Plan                        may play a role in further suppressing the  information  as possible prior to initiating
      The treatment plan included oral hygiene in-  patient’s already compromised immune sys-  treatment. Invasive procedures must be ex-
      structions, full mouth scaling and root plan-  tem. Further, it is important not to prescribe  ecuted  cautiously, with careful  soft tissue
      ing and regular maintenance to address his  clindamycin or aspirin in patients with GI  manipulation. Other tools that may be help-
      periodontal condition. The initial treatment  conditions because of their significant gas-  ful in treating patients with bleeding disor-
      was done in two sessions and calculus was  trointestinal side effects. None of the afore-  ders include hemostatic agents like Surgicel
      removed successfully, as confirmed by tac-  mentioned intraoral lesions were detected in  and Gelfoam, antifibrinolytic mouth rinses,
      tile exploration. While bleeding did occur  this patient.                  or stents with tranexamic  acid. 4, 19  An  ex-
      due to the nature of the procedure, hemosta-                               tra-soft toothbrush is recommended to min-
      sis was achieved shortly after treatment was  Hemostatic Modifications     imize gingival bleeding at home.  Previous
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      completed.  Re-evaluation  after  six weeks  HPS patients can present with thrombocy-  literature  has demonstrated  successful im-
      showed significant improvement in the peri-  topathy. Delta granules (dense bodies) are  plant treatment in patients with HPS, but it
      odontal condition, with less bleeding upon
      probing and reduced pocket depths. Figures   Condition             Dental Modification
      1 to 5 demonstrate  the patient’s condition
      before the initiation of treatment.       Ocular Considerations    Protective eyewear
                                                Bleeding and Hemostatic   Clotting ability via PFA-100 test
      DISCUSSION                                Considerations           Presence of dense bodies via electron microscopy
      HPS is a  complex  condition  that  involves                       Avoid prescribing aspirin
      various  organ systems and  may  require                           Close consultation with hematologist for implant surgery
      close  oral  health  monitoring  and  a  wide                      or tissue augmentation
      range  of  dental  treatment  modifications.
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      The patient presented with mild HPS  and   Gastrointestinal Considerations  Increased frequency of oral ulcers, periodontitis and
      did  not  require  modifications  for  his  den-                   higher DMFT score
      tal treatment. However, in order to prevent                        Avoid prescribing clindamycin
      complications, very careful and meticulous   Immune Considerations   ANC <500 cells/µL indicates antibiotic coverage
      scaling and root planing was performed with                        Increase risk of developing pre-malignant conditions
      special attention to avoid soft tissue injury.
      He was instructed to use a soft toothbrush   Renal Considerations  Avoid prescribing NSAIDs
      to minimize any potential gingival irritation   Pulmonary Considerations  Avoid use of rubber dam if breathing is compromised
      and bleeding. 10                                                   Patient to be seated upright

                                               Table 1. Conditions associated with HPS and related dental modifications
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