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PIYALI BHATTACHARYA
1ST YEAR PGT, HIDSAR
PROSTHODONTICS CROWN & BRIDGE
ORAL MANIFESTATIONS OF
HEMATOLOGIC DISORDERS
Significance of Blood Disorder to a dentist ?
• Tells us about the over all health status of the
patient,
• These patients are prone to infection, often
possess low immunity which may lead to failure of
the treatment
• May raise medical emergency
• Presence of blood impedes visibility of the
operating field
INTRODUCTION
Disorders of RBCs
1. Anemia
• Iron Deficiency Anemia
• Sickle Cell Anemia
• Thalassemia
• Megaloblastic Anemia
• Pernicious Anemia
• Aplastic Anemia
2. Polycythemia
Iron Deficiency Anemia
Oral Manifestation:
Pallor of the mucosa
Generalized atrophy of oral mucosa
Tongue changes: pallor of tongue
with soreness or burning sensation.
Atrophy of filliform papillae over
anterior 2/3 of tongue. Tongue is
Smooth, waxy.
Plummer-Vinson / Paterson-Kelly-Brown Syndrome
characterized by difficulty in swallowing, iron-deficiency
anemia
Oral Manifestations:-
Glossitis Cheilosis Esophgeal Varices
Oral Manifestation:
• Blue, Brown or green hue on teeth
due to deposition of blood
metabolites on enamel and dentin
• Enamel hypoplasia in cases of
erythroblastosis fetalis
• Rh Hump: ring like defects on
teeth
Erythroblastosis Foetalis
Sickle Cell Anemia
Oral Manifestation :
• Pallor and jaundice
• Delayed eruption of teeth and hypoplasia
• These patients are prone to develop osteomyelitis due to
hypovascularity of bone marrow secondary to thrombosis.
• Jaw bones: Mongoloid facies with high cheek bones,
bimaxillary prognathism due to marrow hypoplasia resulting
in an increase in hard palate length and palate alveolar ridge
angle.
Oral Manifestation:
Excessive overgrowth of maxilla with excessive lacrimation
and nasal stuffiness
• Oral mucosa: pale oral mucosa with lemon yellow tint due
to chronic jaundice,
• Rodent facies: marked over development of maxilla
associated with hyperplasia of aveolar process resulting in
anterior open bite with prominent cheek bones .
Thalassemia
• Chipmunk Facies:
Saddle nose,
prominent malar bones and
pneumatization of maxillary sinus.
• Teeth: protruded maxillary teeth with
spacing between them, discoloration of
enamel and dentin due to deposition of
iron.
• Lip: short upper lip due to lag between
growth of maxilla and growth of upper lip.
Oral Manifestation:
Burning sensation of tongue, hypersensitivity, paresthesia and
later dryness of mouth
Hunter’s glossitis: Atrophy of filliform
and fungiform papillae leads to smooth
surface of tongue
Angular cheilitis and dysphagia
Yellowish/ brown pigmentation of oral
Mucosa due to bile pigment deposition.
Megaloblastic Anemia
Oral Manifestation:
• Beefy red tongue, Hunter’s Glossitis
with “Bald Tongue of Sandwith ”
appearance as seen in Pellegra.
• Xerostomia , greenish yellow
pigmentation of oral mucosa
Pernicious Anemia
Oral Manifestation:
• Gingival hyperplasia
• Petechie on soft palate,
ulceration with gray or
black membrane.
• Increased tendency to bleed
and ulceration
Aplastic Anemia
Oral manifestation:
• Erythema
• Glossitis
• Erythematous and edematous gingiva
• Spontaneous bleeding from gingiva
Oral health consideration:
• No established guidelines for delivering dental care to
polycythemia patients
• Poorly controlled patients increased risk of thrombosis and
haemorrhage due to hypercoagulability and quantitative and
qualitative platelet alterations.
Polycythemia
Haemophilia A & B :
• Due to deficiency of F VIII and F IX respectively
• Inherited as an X linked recessive trait. i.e.; only males are
affected.
• In case of hemophilia A, mild symptoms such as prolonged
bleeding following extraction, surgical procedures or severe
trauma occur only if level of F VIII is below 6%-50% .
• Oral manifestation:
Gingival bleeding, bleeding in the floor of the mouth,
Physiological process of tooth eruption may cause severe
bleeding
Clotting Disorders
Prevention
1. Tooth brushing
2. Diet
3. Regular dental care
Dental Procedures
Local anesthesia
Buccal infiltration: A standard agent such as lidocaine with adrenaline
The buccal bone surrounding the lower molar teeth is denser and
does not allow the local anesthetic to infiltrate adequately. Articaine,
an amide anesthetic, has the ability to penetrate the buccal bone using
a standard infiltration technique. the patient should be left for 5-10
minutes before carrying out any treatment in order to achieve
adequate anesthesia.
Intrapapillary injections
Intraligamentary injections
Dental Management
Dental Extractions:
• The patient should be observed for 24 hours following
the extraction
• Splint is constructed to cover the socket completely
• The tooth should be extracted as atraumatically as
possible
• The splint should then be fitted and left in situ for at
least 48 hours.
• Tranexamic acid tablets (1 g, or 15 mg/kg, every 8 hours)
should ideally be started the day before treatment for a
total of 7 days
After 48 hours, the splint should be removed and the socket
checked.
Infection:
Minimal facial swelling: antibiotics
Marked facial swelling: Dental sepsis, which causes
significant facial swelling, can become life-threatening.
• Autosomal dominant disorder, caused due to F V deficiency.
Oral Manifestation:
• Gingival bleeding
• Impossible to clinically differentiate this disease from
hemophilia
• Lab Finding:
Normal or little elevated bleeding time with infinite clotting
and prothrombin time
Parahemophilia
Hypofibrinogenemia
• Macroglobulinemia :
• Synonym: macroglobulinemia of waldenstrom.
• It is a plasma cell dyscrasia manifested as severe anemia (Hb
level as low as 4%-6%).
Oral Manifestation:
• Gingival bleeding
• Oral ulcer
Bleeding Disorders
Synonym: Osler-Rendu-weber Syndrome after Osler(1901)
Autosomal dominant disorder characterised by bleeding from
mucous membrane and lesions on skin and mucosa due to
permanently enlarged capillaries.
Manifestations:
• Lesions bleed easily on slightest pressure.
• Epistaxis
• Severe bleeding from GI tract
Hereditary Hemorrhagic Telangectasia
• Crushed Spider Appearance:
Lesions are cherry red to
purplish macule or papule
Resembles that of a crushed
Spider.
Oral lesions and severe oral
Bleeding several times a day for week.
Platelet Disorders/Purpura
Quantitative Disorder
• Idiopathic Thrombocytopenic
• Secondary Thrombocytopenic
• Congenital
Quanlitative Disorder
• Von Willebrand’s Disease
• Aldrich’s Syndrome
• Bernerd-Soulier Syndrome
Synonym : Primary thrombocytopenic Purpura
• Autoimmune disorder characterized by abnormal decrease
in platelet count in blood(as low as 50000/cu mm.) or
raised number of megakaryocytes in bone marrow.
Clinical Features
• Petechiae, Ecchymosis, Hematoma, epistaxis, Hematuria,
Malena
• Intracranial hemorrhage : mostly seen in children
Oral Manifestation
• Bleeding after tooth extraction
• Gingival bleeding
Idiopathic Thrombocytopenic Purpura
Submucous petechiae, echhymosis, petechiae on tongue
Petechiae at the junction of hard
and soft palate
Synonym : Pseudohaemophilia / vascular haemophilia/ vascular
purpura
Inherited as an autosomal dominant disorder trait with defect in all
three components of haemostatic mechanism- capillaries, platelets,
coagulation mechanism(quantitative and qualitative disorder of
F VIII).
• Characterised by bleeding tendencies gingival bleeding
• Avoid prescribing Aspirin
Von willebrand’s Disease
Cyclic Neutropenia
Symptoms: Fever, sore throat, stomatitis, regional
lymphadenopathy, headache, cutaneous lesion, arthritis,
conjunctivitis, upper respiratory tract infection, lung infection.
Oral manifestation:
Lesions around lip, tongue, palate, gum, buccal mucosa
Gingivitis
Ulceration
Disease of WBC
Clinical Features:
Albinism, recurrent infection, hepatosplenomegaly,
Cervical lymphadenopathy,
Malignant lymphoma
Oral Manifestation:
Ulceration, gingivitis, periodontitis, glossitis
Chediak-Higashi Syndrome:
Leukemia is the proliferation of a clone of abnormal
hematopoietic cells with impaired differentiation, regulation, and
programmed cell death.
Classification
Leukemia
Acute Chronic
Myeloid
Myeloid
Lymphoid
Lymphoid
Affects at early childhood < 5 yrs , the risk declines until mid 20s,
again rises after 50 yrs of age.
Clinical Features:
Weight loss, unexplained fever, Lymphadenopathy, joint pain,
muscle pain, fatigue, malaise, anemia/pallor, mucosal
bleeding, petechie, local infection.
Oral manifestation:
Laryngeal pain, gingival bleeding, gingival enlargement,
gingival ulceration, oral infection,
Acute Lymphocytic Leukemia
Increased rate of caries, recurrent herpes infection, mucosal pallor,
dryness of lip, mucosal petechie, ecchymosis .
The type of leukemia, gender, phase of the chemotherapy
determines the oral condition of the patient.
Management:
Chemotherapy
Allogenic hematopoietic stem cell transplantation recommended only
in suitable donor.
Acute Myeloid Leukemia
Clinical Features and oral manifestations are same as that of ALL.
The management of acute leukemia is divided into three phases:
A) Remission Induction: the bulk of tumour is destroyed by
combination radiotherapy
B) Remission Consolidation: If patient responds to remission
therapy, residual tumour is attacked by consolidation therapy.
C) Remission Maintenance: If patient is responding to
consolidation therapy for acute lymphoblastic tumour a period
of maintenance therapy is given.
Presents with less pronounced marrow failure than acute leukaemia
Average age at which the disease occurs is about 65 yrs.
Cause:
Increased age,
Exposure to ionizing radiation and benzene,
Pathological features:
Myeloid marrow hyperplasia with increased myeloid cell in the blood
and translocation of chromosome t (9, 22)
.
Chronic Myelogenous Leukaemia
Clinical Features:
A chronic indolent course of 3-5 yrs. Followed by an accelerated
phase and blast crisis resembling acute leukaemia .
In the acute/blast phase , the most common manifestations are fever,
weakness, fatigue, anorexia, weight loss, splenomegaly, anaemia,
infection.
Treatment:
Preblast phase treatment is palliative.
Firstline therapy is imatinib,
Stem cell transfer for patients who progress from chronic phase to
blast phase and patients who present in the blast phase.
Results from slow accumulation of clonal B lymphocytes.
Average age of onset is about 65 yrs.
There are two kinds of CLL- one that has a survival rate of 15 yrs.
Hence, it hardly needs to be treated.
Other one having faster progression and a survival rate of 8 yrs.
The staging is based on the involvement of liver, spleen, lymphnodes,
anemia, thrombocytopenia etc.
Chronic Lymphocytic Leukaemia:
Clinical Features:
Symptoms: Fever, lymphadenopathy, fatigue, night sweats, weight
loss, anemia, thrombocytopenia.
Oral manifestation:
Incidence rate of oral manifestation increases once chemotherapy
is started.
Leukemoid Reaction
When the peripheral blood picture in non-leukemic patients resembles
that of leukemia.
Absolute neutrophil count remains above 30,000/ cu.mm.
Subleukemia :
When the total WBC is normal and leukemic cells are found in the
peripheral blood.
Aleukemia:
When no abnormal leukocytes are seen in the peripheral blood and
can only be found in the bone marrow.
Oral health consideration of leukemic patients(Children):
1.) Oral management before leukemic treatment
2.) Oral management during leukemic treatment
3.) Oral management after leukemic treatment
Solid tumours of the immune system.
It has a bimodal age of occurrence . usually affects people from
higher socio-economic background.
Classification:
WHO Classification:
1. Nodular sclerosis (60-80%),
2. Mixed cellularity (15-30%),
3. Lymphocyte depletion (<1%),
4. Lymphocyte rich (5%).
Hodgkin’s Lymphoma
Pathognomic features:
Effacement of lymphnode with destruction of its normal structure,
inflammatory infiltrate of histiocyte, lymphocytes, plasma cells,
fibroblasts and reed Sternberg cells.
Clinical features:
• Asymptomatic enlargement of supradiaphragmatic lymph nodes . commonly,
mediastenial lymphnodes involvement which may cause chest pain, dyspnea,
cough, in some cases, involvement of waldeyer’s ring structures.
• Systemic symptoms: fever that comes and goes for days to weeks, drenching
night sweats ,
• Weight loss (about 10% of body weight in 6 months), generalized pruritus
Treatment:
A) Chemotherapy with ABVD (Adriamycin, Bleomycin,
Vinblastin, Dacarbazine) – gives better responses
B) Chemotherapy with MOPP (Mechloramine, vincristine,
procarbazine, prednisolone)- has many side effects such as
Leukemis, lung cancer, sterility.
C) Radiation Therapy
D) Combination of all of these.
Oral health consideration:
• The radiation fields for HL that involve bilateral cervical
nodes have the potential to result in damage to the salivary
glands
• The mini or modified mantle radiation therapy does not
involve the parotid gland .So the risk of radiation caries is
lessened. However topical fluoride varnish, gel or tooth
paste can be used for caries prevention.
Associated with chronic inflammatory disease such as
rheumatoid disease, Sjogren’s syndrome, celiac disease.
Clinical features:
• Shortness of breath, swollen abdomen, lymphadenopathy,
fever, weight loss, night sweats, fatigue, severe or frequent
infection, .
• Bone marrow involvement may be there along with
cytopenias, easy bruising/ bleeding,
• Aggressive lymphomas may present with abdominal mass/
mediastenial mass.
Non-Hodgkin’s Lymphoma:
• Oral Manifestations:
• Rare for primary NHL but may present as gingival or mucosal
swelling or mass.
• May manifest as intra-bony involvement characterized by
osseous rarefaction around roots of asymptomatic teeth,
mimicking toothache. In such cases, rapid growth from non-
healing extraction site
• Nerve invasion can give rise to paraesthesia or anaesthesia of
the oral structure.
• Non healing ulcer of oral mucosa with ill-defined border,
lesions resembling vesiculobullous disease.
Treatment:
• NHL is a radiosensitive tumor .
• For indolent tumor or low grade tumor 24 Gray
radiotherapy, for aggressive tumor 30 Gray radiotherapy
to consolidate chemotherapy or as a palliative therapy.
A highly aggressive , small, noncleaved cell lymphoma and it was
the first tumor associated with EBV infection.
Clinical Features:
• Affects jaw bones kidneys, GI tracts and other extra nodal
sites.
• Patients may have malignant pleural effusion or ascites and
involvement of the lymph nodes, jaws, ovaries, kidneys,
breasts, omentum, waldeyer’s ring etc.
Burkitt’s Lymphoma:
Oral manifestation:
Clinically evident tumor that may result in tooth mobility, pain,
intraoral swelling of the mandible and maxilla and anterior
open bite, resorption of jaw bones,
Radiographic findings:
Include radiolucent images of bone destruction with poorly defined and irregular
margins
Treatment :
• Children with localized disease, surgical removal with two cycles
of moderately intensive chemotherapy .
• Children with residual stage III disease need four cycles of dose-
intensive chemotherapy.
• Chemotherapy with vincristine, doxorubicin and dexamethasone
or dexamethasone plus thalidomide.
• To manage bony lytic lesion radiation therapy, surgery and
analgesics are used.
A neoplastic plasma cell disorder characterized by clonal plasma
cell proliferation in the bone marrow microenvironment,
monoclonal protein in the blood or urine and associated organ
dysfunction.
Diagnostic criteria:
End organ damage, the presence of at least 10% plasma cells
on examination of the bone marrow and monoclonal protein in
the urine.
Multiple Myeloma:
Clinical manifestation:
• Fatigue, weakness, weight loss, bone pain, recurrent
infection.
• High capacity to induce focal osteolytic bone lesions, diffuse
osteopenia and pathologic fractures.
Oral manifestation :
• Soft tissue masses that are extramedullary plasmablastic
tumors of the jaws(usually in the mandible). Initially
manifested as pain, paraesthesia of the inferior alveolar and
mental nerves, swelling , tooth mobility and radiolucency.
Radiographic changes include typical “Punched-out lesion” in the skull.
Oral Health Considerations:
• Osteoradionecrosis of the jaws resulting in symptomatic
exposed non-healing areas of the maxilla and mandible is
a serious complication of the long-term intravenous
bisphosphonate therapy.
Dental Considerations:
• Dental radiographs should be taken before patients are
placed on intravenous bisphosphonate therapy.
• Scaling and oral prophylaxis should be accomplished as
atraumatically as possible.
• Routine restorative treatment is carried out. Endodontic
treatment, crown , overdenture fabrication is considered
• Dental extractions are avoided unless teeth are grade 3
mobile. Also, extractions are carried out as atraumatically as
possible. Patient is followed up for 4 weeks and then monthly
once until socket is completely healed and closed.
• Re-evaluation of existing prosthetic appliance to make sure
they fit well.
1. Derossi And Raghavendra, Anemia, (Oral Surg Oral Med Oral Pathol
Oral Radiol Endod 2003;95:131-41).
2. Andrew Brewer, Dental Management Of Patients With Inhibitors To
Factor VIII Or Factor IX, April 2008, No:45
3. Deirdre R. Sam et. al. , Managing the dental patient with sickle cell
anemia: a review of the literature, The American Academy of
pediatric Dentistry, Volume 12, Number 5,316-19
4. Joel B. Epstein, Bleeding Disorders of Importance in Dental Care and
Related Patient Management, February 2007, Vol. 73, No. 1
5. Burket’s Oral Medicine, 12th Edition, Michael Glick
6. Davidson’s Principles & Practice of Medicine, 20th Edition
7. Dental considerations for leukemic pediatric patients: an updated review
for general dental practitioner, Mater Sociomed. 2015 Oct; 27(5): 359-
362 • Review
8. Oral Burkitt's Lymphoma - Case Report, Brazilian Journal of
Otorhinolaryngology 74 (3) May/June 2008
References

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Oral manifestation of bleeding disorders

  • 1. PIYALI BHATTACHARYA 1ST YEAR PGT, HIDSAR PROSTHODONTICS CROWN & BRIDGE ORAL MANIFESTATIONS OF HEMATOLOGIC DISORDERS
  • 2. Significance of Blood Disorder to a dentist ? • Tells us about the over all health status of the patient, • These patients are prone to infection, often possess low immunity which may lead to failure of the treatment • May raise medical emergency • Presence of blood impedes visibility of the operating field INTRODUCTION
  • 3. Disorders of RBCs 1. Anemia • Iron Deficiency Anemia • Sickle Cell Anemia • Thalassemia • Megaloblastic Anemia • Pernicious Anemia • Aplastic Anemia 2. Polycythemia
  • 4. Iron Deficiency Anemia Oral Manifestation: Pallor of the mucosa Generalized atrophy of oral mucosa Tongue changes: pallor of tongue with soreness or burning sensation. Atrophy of filliform papillae over anterior 2/3 of tongue. Tongue is Smooth, waxy.
  • 5. Plummer-Vinson / Paterson-Kelly-Brown Syndrome characterized by difficulty in swallowing, iron-deficiency anemia Oral Manifestations:- Glossitis Cheilosis Esophgeal Varices
  • 6. Oral Manifestation: • Blue, Brown or green hue on teeth due to deposition of blood metabolites on enamel and dentin • Enamel hypoplasia in cases of erythroblastosis fetalis • Rh Hump: ring like defects on teeth Erythroblastosis Foetalis
  • 7. Sickle Cell Anemia Oral Manifestation : • Pallor and jaundice • Delayed eruption of teeth and hypoplasia • These patients are prone to develop osteomyelitis due to hypovascularity of bone marrow secondary to thrombosis. • Jaw bones: Mongoloid facies with high cheek bones, bimaxillary prognathism due to marrow hypoplasia resulting in an increase in hard palate length and palate alveolar ridge angle.
  • 8. Oral Manifestation: Excessive overgrowth of maxilla with excessive lacrimation and nasal stuffiness • Oral mucosa: pale oral mucosa with lemon yellow tint due to chronic jaundice, • Rodent facies: marked over development of maxilla associated with hyperplasia of aveolar process resulting in anterior open bite with prominent cheek bones . Thalassemia
  • 9. • Chipmunk Facies: Saddle nose, prominent malar bones and pneumatization of maxillary sinus. • Teeth: protruded maxillary teeth with spacing between them, discoloration of enamel and dentin due to deposition of iron. • Lip: short upper lip due to lag between growth of maxilla and growth of upper lip.
  • 10. Oral Manifestation: Burning sensation of tongue, hypersensitivity, paresthesia and later dryness of mouth Hunter’s glossitis: Atrophy of filliform and fungiform papillae leads to smooth surface of tongue Angular cheilitis and dysphagia Yellowish/ brown pigmentation of oral Mucosa due to bile pigment deposition. Megaloblastic Anemia
  • 11. Oral Manifestation: • Beefy red tongue, Hunter’s Glossitis with “Bald Tongue of Sandwith ” appearance as seen in Pellegra. • Xerostomia , greenish yellow pigmentation of oral mucosa Pernicious Anemia
  • 12. Oral Manifestation: • Gingival hyperplasia • Petechie on soft palate, ulceration with gray or black membrane. • Increased tendency to bleed and ulceration Aplastic Anemia
  • 13. Oral manifestation: • Erythema • Glossitis • Erythematous and edematous gingiva • Spontaneous bleeding from gingiva Oral health consideration: • No established guidelines for delivering dental care to polycythemia patients • Poorly controlled patients increased risk of thrombosis and haemorrhage due to hypercoagulability and quantitative and qualitative platelet alterations. Polycythemia
  • 14. Haemophilia A & B : • Due to deficiency of F VIII and F IX respectively • Inherited as an X linked recessive trait. i.e.; only males are affected. • In case of hemophilia A, mild symptoms such as prolonged bleeding following extraction, surgical procedures or severe trauma occur only if level of F VIII is below 6%-50% . • Oral manifestation: Gingival bleeding, bleeding in the floor of the mouth, Physiological process of tooth eruption may cause severe bleeding Clotting Disorders
  • 15. Prevention 1. Tooth brushing 2. Diet 3. Regular dental care Dental Procedures Local anesthesia Buccal infiltration: A standard agent such as lidocaine with adrenaline The buccal bone surrounding the lower molar teeth is denser and does not allow the local anesthetic to infiltrate adequately. Articaine, an amide anesthetic, has the ability to penetrate the buccal bone using a standard infiltration technique. the patient should be left for 5-10 minutes before carrying out any treatment in order to achieve adequate anesthesia. Intrapapillary injections Intraligamentary injections Dental Management
  • 16. Dental Extractions: • The patient should be observed for 24 hours following the extraction • Splint is constructed to cover the socket completely • The tooth should be extracted as atraumatically as possible • The splint should then be fitted and left in situ for at least 48 hours. • Tranexamic acid tablets (1 g, or 15 mg/kg, every 8 hours) should ideally be started the day before treatment for a total of 7 days
  • 17. After 48 hours, the splint should be removed and the socket checked. Infection: Minimal facial swelling: antibiotics Marked facial swelling: Dental sepsis, which causes significant facial swelling, can become life-threatening.
  • 18. • Autosomal dominant disorder, caused due to F V deficiency. Oral Manifestation: • Gingival bleeding • Impossible to clinically differentiate this disease from hemophilia • Lab Finding: Normal or little elevated bleeding time with infinite clotting and prothrombin time Parahemophilia Hypofibrinogenemia
  • 19. • Macroglobulinemia : • Synonym: macroglobulinemia of waldenstrom. • It is a plasma cell dyscrasia manifested as severe anemia (Hb level as low as 4%-6%). Oral Manifestation: • Gingival bleeding • Oral ulcer Bleeding Disorders
  • 20. Synonym: Osler-Rendu-weber Syndrome after Osler(1901) Autosomal dominant disorder characterised by bleeding from mucous membrane and lesions on skin and mucosa due to permanently enlarged capillaries. Manifestations: • Lesions bleed easily on slightest pressure. • Epistaxis • Severe bleeding from GI tract Hereditary Hemorrhagic Telangectasia
  • 21. • Crushed Spider Appearance: Lesions are cherry red to purplish macule or papule Resembles that of a crushed Spider. Oral lesions and severe oral Bleeding several times a day for week.
  • 22. Platelet Disorders/Purpura Quantitative Disorder • Idiopathic Thrombocytopenic • Secondary Thrombocytopenic • Congenital Quanlitative Disorder • Von Willebrand’s Disease • Aldrich’s Syndrome • Bernerd-Soulier Syndrome
  • 23. Synonym : Primary thrombocytopenic Purpura • Autoimmune disorder characterized by abnormal decrease in platelet count in blood(as low as 50000/cu mm.) or raised number of megakaryocytes in bone marrow. Clinical Features • Petechiae, Ecchymosis, Hematoma, epistaxis, Hematuria, Malena • Intracranial hemorrhage : mostly seen in children Oral Manifestation • Bleeding after tooth extraction • Gingival bleeding Idiopathic Thrombocytopenic Purpura
  • 24. Submucous petechiae, echhymosis, petechiae on tongue Petechiae at the junction of hard and soft palate
  • 25. Synonym : Pseudohaemophilia / vascular haemophilia/ vascular purpura Inherited as an autosomal dominant disorder trait with defect in all three components of haemostatic mechanism- capillaries, platelets, coagulation mechanism(quantitative and qualitative disorder of F VIII). • Characterised by bleeding tendencies gingival bleeding • Avoid prescribing Aspirin Von willebrand’s Disease
  • 26. Cyclic Neutropenia Symptoms: Fever, sore throat, stomatitis, regional lymphadenopathy, headache, cutaneous lesion, arthritis, conjunctivitis, upper respiratory tract infection, lung infection. Oral manifestation: Lesions around lip, tongue, palate, gum, buccal mucosa Gingivitis Ulceration Disease of WBC
  • 27. Clinical Features: Albinism, recurrent infection, hepatosplenomegaly, Cervical lymphadenopathy, Malignant lymphoma Oral Manifestation: Ulceration, gingivitis, periodontitis, glossitis Chediak-Higashi Syndrome:
  • 28. Leukemia is the proliferation of a clone of abnormal hematopoietic cells with impaired differentiation, regulation, and programmed cell death. Classification Leukemia Acute Chronic Myeloid Myeloid Lymphoid Lymphoid
  • 29. Affects at early childhood < 5 yrs , the risk declines until mid 20s, again rises after 50 yrs of age. Clinical Features: Weight loss, unexplained fever, Lymphadenopathy, joint pain, muscle pain, fatigue, malaise, anemia/pallor, mucosal bleeding, petechie, local infection. Oral manifestation: Laryngeal pain, gingival bleeding, gingival enlargement, gingival ulceration, oral infection, Acute Lymphocytic Leukemia
  • 30. Increased rate of caries, recurrent herpes infection, mucosal pallor, dryness of lip, mucosal petechie, ecchymosis . The type of leukemia, gender, phase of the chemotherapy determines the oral condition of the patient. Management: Chemotherapy Allogenic hematopoietic stem cell transplantation recommended only in suitable donor. Acute Myeloid Leukemia Clinical Features and oral manifestations are same as that of ALL.
  • 31. The management of acute leukemia is divided into three phases: A) Remission Induction: the bulk of tumour is destroyed by combination radiotherapy B) Remission Consolidation: If patient responds to remission therapy, residual tumour is attacked by consolidation therapy. C) Remission Maintenance: If patient is responding to consolidation therapy for acute lymphoblastic tumour a period of maintenance therapy is given.
  • 32. Presents with less pronounced marrow failure than acute leukaemia Average age at which the disease occurs is about 65 yrs. Cause: Increased age, Exposure to ionizing radiation and benzene, Pathological features: Myeloid marrow hyperplasia with increased myeloid cell in the blood and translocation of chromosome t (9, 22) . Chronic Myelogenous Leukaemia
  • 33. Clinical Features: A chronic indolent course of 3-5 yrs. Followed by an accelerated phase and blast crisis resembling acute leukaemia . In the acute/blast phase , the most common manifestations are fever, weakness, fatigue, anorexia, weight loss, splenomegaly, anaemia, infection. Treatment: Preblast phase treatment is palliative. Firstline therapy is imatinib, Stem cell transfer for patients who progress from chronic phase to blast phase and patients who present in the blast phase.
  • 34. Results from slow accumulation of clonal B lymphocytes. Average age of onset is about 65 yrs. There are two kinds of CLL- one that has a survival rate of 15 yrs. Hence, it hardly needs to be treated. Other one having faster progression and a survival rate of 8 yrs. The staging is based on the involvement of liver, spleen, lymphnodes, anemia, thrombocytopenia etc. Chronic Lymphocytic Leukaemia:
  • 35. Clinical Features: Symptoms: Fever, lymphadenopathy, fatigue, night sweats, weight loss, anemia, thrombocytopenia. Oral manifestation: Incidence rate of oral manifestation increases once chemotherapy is started.
  • 36. Leukemoid Reaction When the peripheral blood picture in non-leukemic patients resembles that of leukemia. Absolute neutrophil count remains above 30,000/ cu.mm. Subleukemia : When the total WBC is normal and leukemic cells are found in the peripheral blood. Aleukemia: When no abnormal leukocytes are seen in the peripheral blood and can only be found in the bone marrow.
  • 37. Oral health consideration of leukemic patients(Children): 1.) Oral management before leukemic treatment 2.) Oral management during leukemic treatment 3.) Oral management after leukemic treatment
  • 38. Solid tumours of the immune system. It has a bimodal age of occurrence . usually affects people from higher socio-economic background. Classification: WHO Classification: 1. Nodular sclerosis (60-80%), 2. Mixed cellularity (15-30%), 3. Lymphocyte depletion (<1%), 4. Lymphocyte rich (5%). Hodgkin’s Lymphoma
  • 39. Pathognomic features: Effacement of lymphnode with destruction of its normal structure, inflammatory infiltrate of histiocyte, lymphocytes, plasma cells, fibroblasts and reed Sternberg cells. Clinical features: • Asymptomatic enlargement of supradiaphragmatic lymph nodes . commonly, mediastenial lymphnodes involvement which may cause chest pain, dyspnea, cough, in some cases, involvement of waldeyer’s ring structures. • Systemic symptoms: fever that comes and goes for days to weeks, drenching night sweats , • Weight loss (about 10% of body weight in 6 months), generalized pruritus
  • 40. Treatment: A) Chemotherapy with ABVD (Adriamycin, Bleomycin, Vinblastin, Dacarbazine) – gives better responses B) Chemotherapy with MOPP (Mechloramine, vincristine, procarbazine, prednisolone)- has many side effects such as Leukemis, lung cancer, sterility. C) Radiation Therapy D) Combination of all of these.
  • 41. Oral health consideration: • The radiation fields for HL that involve bilateral cervical nodes have the potential to result in damage to the salivary glands • The mini or modified mantle radiation therapy does not involve the parotid gland .So the risk of radiation caries is lessened. However topical fluoride varnish, gel or tooth paste can be used for caries prevention.
  • 42. Associated with chronic inflammatory disease such as rheumatoid disease, Sjogren’s syndrome, celiac disease. Clinical features: • Shortness of breath, swollen abdomen, lymphadenopathy, fever, weight loss, night sweats, fatigue, severe or frequent infection, . • Bone marrow involvement may be there along with cytopenias, easy bruising/ bleeding, • Aggressive lymphomas may present with abdominal mass/ mediastenial mass. Non-Hodgkin’s Lymphoma:
  • 43. • Oral Manifestations: • Rare for primary NHL but may present as gingival or mucosal swelling or mass. • May manifest as intra-bony involvement characterized by osseous rarefaction around roots of asymptomatic teeth, mimicking toothache. In such cases, rapid growth from non- healing extraction site • Nerve invasion can give rise to paraesthesia or anaesthesia of the oral structure. • Non healing ulcer of oral mucosa with ill-defined border, lesions resembling vesiculobullous disease.
  • 44. Treatment: • NHL is a radiosensitive tumor . • For indolent tumor or low grade tumor 24 Gray radiotherapy, for aggressive tumor 30 Gray radiotherapy to consolidate chemotherapy or as a palliative therapy.
  • 45. A highly aggressive , small, noncleaved cell lymphoma and it was the first tumor associated with EBV infection. Clinical Features: • Affects jaw bones kidneys, GI tracts and other extra nodal sites. • Patients may have malignant pleural effusion or ascites and involvement of the lymph nodes, jaws, ovaries, kidneys, breasts, omentum, waldeyer’s ring etc. Burkitt’s Lymphoma:
  • 46. Oral manifestation: Clinically evident tumor that may result in tooth mobility, pain, intraoral swelling of the mandible and maxilla and anterior open bite, resorption of jaw bones,
  • 47. Radiographic findings: Include radiolucent images of bone destruction with poorly defined and irregular margins
  • 48. Treatment : • Children with localized disease, surgical removal with two cycles of moderately intensive chemotherapy . • Children with residual stage III disease need four cycles of dose- intensive chemotherapy. • Chemotherapy with vincristine, doxorubicin and dexamethasone or dexamethasone plus thalidomide. • To manage bony lytic lesion radiation therapy, surgery and analgesics are used.
  • 49. A neoplastic plasma cell disorder characterized by clonal plasma cell proliferation in the bone marrow microenvironment, monoclonal protein in the blood or urine and associated organ dysfunction. Diagnostic criteria: End organ damage, the presence of at least 10% plasma cells on examination of the bone marrow and monoclonal protein in the urine. Multiple Myeloma:
  • 50. Clinical manifestation: • Fatigue, weakness, weight loss, bone pain, recurrent infection. • High capacity to induce focal osteolytic bone lesions, diffuse osteopenia and pathologic fractures. Oral manifestation : • Soft tissue masses that are extramedullary plasmablastic tumors of the jaws(usually in the mandible). Initially manifested as pain, paraesthesia of the inferior alveolar and mental nerves, swelling , tooth mobility and radiolucency.
  • 51. Radiographic changes include typical “Punched-out lesion” in the skull.
  • 52. Oral Health Considerations: • Osteoradionecrosis of the jaws resulting in symptomatic exposed non-healing areas of the maxilla and mandible is a serious complication of the long-term intravenous bisphosphonate therapy.
  • 53. Dental Considerations: • Dental radiographs should be taken before patients are placed on intravenous bisphosphonate therapy. • Scaling and oral prophylaxis should be accomplished as atraumatically as possible. • Routine restorative treatment is carried out. Endodontic treatment, crown , overdenture fabrication is considered
  • 54. • Dental extractions are avoided unless teeth are grade 3 mobile. Also, extractions are carried out as atraumatically as possible. Patient is followed up for 4 weeks and then monthly once until socket is completely healed and closed. • Re-evaluation of existing prosthetic appliance to make sure they fit well.
  • 55. 1. Derossi And Raghavendra, Anemia, (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:131-41). 2. Andrew Brewer, Dental Management Of Patients With Inhibitors To Factor VIII Or Factor IX, April 2008, No:45 3. Deirdre R. Sam et. al. , Managing the dental patient with sickle cell anemia: a review of the literature, The American Academy of pediatric Dentistry, Volume 12, Number 5,316-19 4. Joel B. Epstein, Bleeding Disorders of Importance in Dental Care and Related Patient Management, February 2007, Vol. 73, No. 1 5. Burket’s Oral Medicine, 12th Edition, Michael Glick 6. Davidson’s Principles & Practice of Medicine, 20th Edition 7. Dental considerations for leukemic pediatric patients: an updated review for general dental practitioner, Mater Sociomed. 2015 Oct; 27(5): 359- 362 • Review 8. Oral Burkitt's Lymphoma - Case Report, Brazilian Journal of Otorhinolaryngology 74 (3) May/June 2008 References