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1. Dental Management Of Patient With
Thalassemia
PRESENTED BY
DR. IBRAHIM MUNEIM HUSSEIN
SUPERVISED BY
DR. SALLY TALIB

2. WHAT IS
ANAEMIA ?

3.  Thalassemia is an autosomal recessive genetic disorder characterized by a
reduced rate of production of alpha or beta globulin chains in hemoglobin
molecules, thus resulting in the production of faulty hemoglobin.
 Depending on the globin chain affected, thalassemia can be divided into two
major groups: Alpha thalassemia and Beta thalassemia
 Word Thalassemia is derived from the Greek word Thalass ("sea") and -emia
("blood").
 The Disease is very common in peoples living in Mediterranean geographical
region, but found all over the world .
 The disease usually occurs in children aged only a few months.
Introduction

4. ALPHA (α) THALASSEMIA
 The α-thalassemia involve the genes HBA1 and HBA2, inherited in a Mendalian
fashion.
 It is also connected to the deletion of the chromosome 16.
 There are two gene loci and so four alleles
 α Thalassemia result in decreased alpha-globin production, therefore fewer alpha-
globin chains are produced, resulting in an excess of β chains in adults and excess γ
chains in newborns.

5. BETA (β)THALASSEMIA
 Beta(β)thalassemia are due to mutations in the HBA1 gene on chromosome 11,
inherited in an autosomal recessive fashion.
 Beta thalassemia is classified into two categories βo or β+ ; beta plus, where the beta
chain production is reduced; and beta zero where there is no beta chain production
found.
 This beta zero thalassemia is known as beta major thalassemia and beta plus
thalassemia is known as intermediate beta thalassemia.
 Beta major thalassemia is a homozygote beta thalassemia disorder that is often called
Cooley anemia. Its clinical manifestation usually appears after 4-6 months of life and the
patients experience severe anemia with less than 20% hematocrit that leads to
dependency towards blood transfusion

6. The heterozygous form is called minor thalassemia or thalassemia trait; it
comes with limited clinical signs and may be confused with iron deficiency
anemia.
In the homozygous form, two groups can be distinguished based on whether or
not regular blood transfusions are required: beta thalassemia intermedia and
beta thalassemia major.
Patients with Thalassemia Intermedia have moderate anemia. Due to the
presence of a higher amount of healthy hemoglobin, these patients do not need
blood transfusion on the regular. Their prognosis therefore is much better.

7. Oro-facial features
 Skeletal class II malocclusion due to the protrusion of the maxillary and
general resistance of the mandible to expansion
 Major mandibular transverse defects (Brodie syndrome)
 Increased overjet
 Anterior open bite
 Malar prominence, saddle nose, frontal bossing (Chipmunk facies)
 Orbital hypertelorism
 Migration and spacing of upper teeth
 X - ray findings include rarefaction of the alveolar bone, thinning of the
mandibular cortex, obliteration of paranasal sinuses, short roots,
taurodontism, thinning of the lamina dura.

8. Increase in dental decay
Delayed dental development
Change in dental morphology
Mucosal Pallor and dental discolouration
Alveolar bone may have a ‘chicken wire-like’ radiological
appearance
Delayed pneumatisation of maxillary sinuses
Painful swelling of parotids and xerostomia (due to iron deposits)
Sore or burning tongue due to folate deficiency
Oral ulceration (very rare)
Necrotizing gingivostomatitis (very rare)

9. Profile view of a 13–year-old boy with
thalassaemia major showing typical facial
features of thalassemia major;
characterized by frontal bossing, bulging
cheekbone, saddle nose, and protrusive
premaxilla.

10.  patients commonly show a typical face
called facies Colley with abnormal
facial and cranial bone growth.
 The upper jaw seems to be bigger due to
the bone marrow expansion. This facial
feature is known as chipmunk facies.

11.  Hair on end appearance in the cranium
(crew cut appearance )
 Honeycomb appearance in the panoramic
image.

12.  Cephalometric radiograph of a 15-year-
old boy with thalassemia major
disclosing prominent premaxilla,
thickened frontal bone, thinned inferior
border of the mandible and partially
obliterated maxillary sinus.

13. Test of Thalassemia
 A complete blood count (CBC) reveals anemia
 Red blood cells will appear small and abnormally shaped when looked at
under a microscope..
 A test called hemoglobin electrophoresis shows the presence of an
abnormal form of hemoglobin.
 A test called mutational analysis can help detect alpha thalassemia that
cannot be seen with hemoglobin electrophoresis.

14. Dental Management
 When a patient of thalassemia came for Dental Treatment, it is necessary that
maximum information about patient has to be taken.
 Detailed history
 Examination (systemic and Oral examination )
 Medical Diagnosis of Thalassemia
 Life expectancy
 Condition of Patient
 Coagulation and vitamin k Deficiency

15. Possibility of orthodontic and/or orthopedic treatments? Maxillary osteotomies are not
indicated for beta thalassemia major patients particularly if blood transfusion and chelator
therapy has been shown to be barely effective It is recommended to initiate preventive and
interceptive orthodontics as early as possible to correct anterior maxillary teeth drifting and
the excessive overjet and to reduce the susceptibility of trauma.
Forces used in thalassemic patients should be low since there is an increased risk of fracture
due to the thin corticals These patients should be followed more closely with shorter
intervals between appointments. The retention phase usually proves more difficult.
Dental Aspect

16. Anemia and hemoglobin levels
Keep the visits short, adapt to patient’s tolerance level, take into
consideration poor healing especially during invasive procedures, patients
should be medically cleared before invasive procedures, their
hemoglobin level should not be lower than 110g/l

17. Iron overload and iron chelation
Dentists should always be aware of iron overload (mild, moderate or
heavy) through biological tests, as it impacts the prognosis. Dentists need
to take additional precautions to compensate for potential complications
such as impaired liver function and diabetes.
Oral chelators’ side effects can include hepatitis, neutropenia, kidney
dysfunction, cytopenia, agranulocytosis and gastrointestinal bleeding
Caution should be exercised while prescribing any medication.
The possible involvement of the immune system as a side effect of oral
chelators is one more argument towards the use of antibiotic coverage.

18. Cardiac involvement
Even asymptomatic cardiac dysfunctions can get complicated under
situations of stress.
Dentists should enquire about any underlying heart ailments or
medication. Anesthetics with vasoconstrictors should be carefully used
with the presence of arrhythmias especially if they’re treated with beta
blockers Epinephrine may raise blood pressure and complicate
arrhythmias and chest pain.
Risk of pericarditis and sepsis should be kept in mind in case of invasive
procedures and periodontal disease.

19. Hepatic manifestation
Presence or not of HVC/HVB should be known. In the case of associated
hepatic disease, caution must be used when prescribing medication, as to
avoid hepatotoxic drugs.

20. Thalassemia patients can present generally mild liver alternations., In extreme cases may
involve strongly diminished coagulation factor production, intense vitamin K absorption
deficiencies due to continuous antibiotic coverage provided, or previously undetected and
progressing hepatitis.
It is also important to exercise caution when using hepatotoxic drugs of common use in
dental practice
The liver function test and coagulation tests(PT,APTT,INR) must be carried out before
treatment.
In thalassemia , several transfusions was given to secure coagulation, especially as a
result of the administered platelet fraction, coagulation tests must be also carried out
before starting dental treatment

21. • This may require extreme care and caution in dental treatments The increase in platelet
count resulting from splenectomy implies a greater risk of thrombosis (local coagulation
or clotting of the blood in a part of the circulatory system).
• The administration of antiplatelet drugs in such cases requires monitoring of bleeding
time or consultation with a hematologist.

22. Splenectomy
Always check for a history of splenectomy as the presence or absence of this
organ changes the dental approach. Due to risks of bacteremia and
sepsis, antibiotic prophylaxis is obligatory, As splenectomised patients are
usually under regular penicillin chemoprophylaxis, a change in antibiotic is
recommended during dental treatment to avoid bacterial resistance.
Thromboembolic complications are more frequent in splenectomised patients,
which may lead to being radical in dental treatments to prevent infection
spread In consultation with the treating hematologist, it is possible to resort to
administration of anti-platelet drugs to reduce the risk of thrombosis.

23. • The absence of hypersplenism without leucopenia (a reduction in the number of white
cells in the blood) or thrombocytopenia (deficiency of platelets in the blood) facilities
dental treatment of both thalassemia major and thalassemia intermediate.
• In contrast the presence of hypersplenism with leucopenia and thrombocytopenia
requires the provision of antibiotic coverage and platelet concentrates before dental
procedures can be carried out.
• In the case of splenectomy patients, the risk of sepsis (the presence in tissues of
harmful bacteria and their toxins, typically through infection of a wound) is high and
the oral cavity must be prevented from constituting a source of bacterial spread.

24. Diabetes
Periodontal disease and gingivitis foci are potential
sources of infection that should alert the dentist to
adopt strict preventive measures.

25. Infections
Guidelines regarding antibiotic prophylaxis vary with some recommending
prophylaxis similar to that used for the prevention of bacterial
endocarditis. Dental infections or abscesses should are an emergency and
should not be left untreated.

26. Invasive procedures should be done in the week after transfusion
following antibiotic prophylaxis and the study of the coagulation profile
Hemoglobin level should be more than 110g/l before any clinical
procedure.
Transfusion therapy schedule

27. Bisphosphonates are commonly used in thalassemia patients to inhibit
bone resorption Comprehensive health and dental assessments should be
carried out before the start of the treatment. Due to the high
risks of bisphosphonate related necrosis of the jaw, it is important to
reduce mucosal trauma and avoid dental extractions.
In case of bone exposure or surgical extractions, the practitioner should
refer the patient to an oral surgery specialist All patients should be
advised of the risk preoperatively and closely monitored post-operatively.
Bisphosphonate therapy

28. The key to optimal dental care for thalassemia patients in general and beta
thalassemia major patients in particular, is multidisciplinary team work.
Close liaison should be maintained with the hematologist and/or
cardiologist during each step of the dental treatment planning.
The more informed the dental practitioner is about this complex condition
and its systemic implications, the better level of care he can provide to his
patients.

29. • It should also be in consideration that dental treatment of thalassemia patients drugs
that have depressive effects upon the central nervous system and hence also upon
respiration, should be used with caution.
• It is also important to taken into consideration that the liver physiology and renal
excretory capacity of these patients are depressed.
Therefore depressant drugs should be avoided or used with care

30. Antibiotics prophylaxis
Terezhalmy and Hall Suggested oral anti microbial regimen for aspleenic (refers to
the absence of normal spleen function) patients undergoing dental or oral surgical
treatments.
• Penicillin V 2 G 30 minutes- 2 hour before procedure, then 500 mg. post operative
q6th for 8 doses.
• Erythromycin 1G 30 minutes to 2hour before procedure, then 500 mg post operative
q6th for 8 doses.
• If the patients are receiving oral deferiprone, the side effects of the drug should be
taken into account, since they can affect already impair organs such as the liver and
immune system.

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