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Influence of Hematological
disorders on the
Periodontium

Dr Saif Khan
Assistant Professor
Dept tof Periodontics & Community Dentistry
Dr Z A Dental College
Aligarh Muslim University, Aligarh,
UP,India-202002
Blood cells play essential role in
maintenance of healthy Periodontium

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• Comparable oral changes occur in more than one
form of blood dyscrasia
• Secondary inflammatory changes produce wide
range of variation in oral signs
• Hemorrhagic tendencies occur when normal
hemostatic mechanisms are disturbed

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• Abnormal Gingival Bleeding or from other areas of
oral mucosa that is difficult to control may suggest
some underlying Hematologic disease or Blood
dyscrasia

• Hemorrhagic tendencies occur when normal
Hemostatic mechanisms are disturbed

• Petechiaes and eechymosis of soft palate may
suggest underlying bleeding disorder
Dr Saif Khan

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It is essential to diagnose the specific
etiology inorder to address any bleeding or
immunologic disorder appropriately

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Leukemia
Malignant neoplasia of WBC precursors
Charcterised by
1.Replacement of Bone marrow with proliferating
cells
2.Abnormal number & form of immature WBCs in
circulating blood
3.Infiltration in liver, spleen, lymph nodes and
other body sites
Classification
According to lineage of WBC, Leukemia are
classified as
• Lymphocytic
• Myelocytic
– Monocytic

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 According to evolution Leukemia can be
– Acute
– Subacute
– Chronic

 Acute Leukemia the primitive blast cells
are released in peripheral circulation
 Chronic anemia the abnormal cells are
more mature with normal morphologic
characteristics and function when released
into the circulation
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Histology AML

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All leukemic cells tend to displace normal
component of Bone marrow elements with
resulting in decreased production of
RBC

Anemia

WBC

Leukopenia

Plateletes

Thrombocytopenia

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Aleukemic Leukemia
• Patients have normal blood counts while
leukemic cells primarily reside in Bone marrow.
• The peripheral blood does not contains any
Leukemic or malignant cell

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Periodontium in Leukemia

• Oral and Periodontal manifestation of
Leukemia consists of
– Leukemic infiltration
– Bleeding or Hemorrhage
– Oral Ulcerations
– Infections

• The expression of above signs are more
common in Acute & Subacute form of
leukemia than Chronic
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Leukemic infiltration
• Leukemic cells infiltrate gingiva and less
frequently alveolar bone
• Gingival infiltration results in Leukemic
Gingival enlargement
• Highest incidence of leukemic gingival
enlargement is found in patients with
– Acute Monocytic Leukemia (66.7%)
– Acute Myelocytic-monocytic leukemia (18.7%)
– Acute Myelocytic leukemia (3.7%)
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• Leukemic gingival enlargement is not
found in edentulous patients or in patients
with chronic leukemia
• Leukemic gingival enlargement consists of
– Infiltration of Gingival Corium by leukemic
cells
– Increased gingival thickness, periodontal
pocket formation, bacterial plaque
accumulation and secondary inflammation

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Clinical features
• Gingiva appears Bluish-red and Cyanotic
• Rounding and Tenseness at Gingival Margin
• Increase in gingival size at interdental papilla
covering the crown of teeth

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Leukemic Gingival Enlargement

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Leukemic Gingival Enlargements

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Microscopically
• Dense,diffuse infiltration of predominantly
immature leukocytes in attached & marginal
gingiva
• Normal connective tissue component of gingiva is
replaced by leukemic cells
• Mitotic figures indicative of ectopic
haemopoiesis may be seen
• Nature of cell infiltrate depends on type of
leukemia

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• Cellular accumulation is denser in entire
reticular connective tissue layer
• Pappillary layer contains comparatively few
leukocytes
• Blood vessels are distended and contain
predominantly leukemic cells
• RBCs are reduced in number
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• Epithelium presents with variety of changes and
may be thinned or Hyperplastic
• Degeneration associated with intercellular and
intracellular edema and leukocyte infiltration
with diminished surface keratinization

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• Microscopic picture of marginal gingiva
differs from other gingival locations
• Consists of notable inflammatory component
in addition to leukemic cells
• Scattered foci of plasma cells and
lymphocytes with edema and degeneration are
common findings
• Inner layer of marginal gingiva is usually
ulcerated with marginal necrosis and
pseudomembrane formation
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• Periodontal ligament and alveolar bone may also
be involved in acute and subacute leukemia
• Periodontal ligament may be infiltrated with
mature and immature leukocytes
• Marrow of alveolar bone exhibits localised area of
necrosis, thrombosis of blood vessels, infilation
with mature and immature leukocytes
• Replacement of fatty marrow with fibrous tissue
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Bleeding
• Oral bleeding has been seen in 17.7% patients
with Acute Leukemia and 4.4% in patient
with Chronic leukemia
• Gingival hemorrhage is common in leukemic
patients
• Bleeding gingiva can be early sign of gingivitis

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• Caused by Thrombocytopenia by replacement
of Bone marrow cells by leukemic cells
• Also by inhibition of stem cell function by
leukemic cells or their products
• Bleeding may be side effect of
Chemotherapeutic agents used to treat
leukemia
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Oral ulceration and Infection
• Granulocytopenia (dimnished WBC count) results
from the displacement of normal bone marrow cells by
leukemic cells
• Discrete, Punched –out ulcers penetrating deeply
into submucosa and covered by a firmly attached
white slough
• Recurrent Herpetic oral ulcers
• Atypical Oral Ulcer
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• Gingiva is peculiar bluish red, is sponge like and friable,
and bleeds persistently on slightest provocation or even
spontaneously in leukemia patients
• Acute gingival necrosis
•

Pseudomembrane formation

• NUG
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• Secondary oral changes superimposed on oral
tissues altered by blood dyscrasia

• Systemic toxic effects, loss of apetite, nausea,
blood loss from persistent gingival bleeding and
constant gnawing pain
• Eliminating or reducing local factors (dental
plaque
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• In chronic leukemia clinical oral changes
suggesting hematologic disturbances are rare

• Gingival biopsy in patient with chronic leukaemia
may reveal typical gingival inflammation without
any suggestion of a hematologic disturbance

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Anemia
• Deficiency in the quantity or quality of the
blood
• Manifested by reduction in the number of
erythrocyte and the amount of hemoglobin
• Anemia results from
– Blood loss
– Defective blood formation
– Increased RBC destruction

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Anemias are classified according to
cellular morphology and hemoglobin content
1. Macrocytic Hyperchromic anemia (Pernicious Anemia)
2. Microcytic Hypochromic anemia (Iron deficiency anemia)
3. Sickle cell anemia
4. Normocytic Normochromic anemia (Hemolytic &Aplastic
anemia)

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Dr Saif Khan

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Sickle Cell Anemia
•
•
•
•

Hereditary
Chronic Hemolytic Anemia
Exclusively in Blacks
Presents with
– Pallor
– Jaundice
– Weakness
– Rheumatoid Manifestations
– Leg Ulcers

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Oral Changes

• Generalised osteoporosis of Jaw
– Stepladder arrangement of trabeculae

• Pallor/Yellowish discoloration
• Periodontal infection may precipitate
Sickle Cell Crisis

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Thrombocytopenia
• Reduced platelet count either due to
– lack of platelet production or
– increased platelet destruction
– Increased loss

• Purpura is
– purplish appearance of skin or mucous membrane that occurs
as a result of decreased platelets

• Thrombocytopenic purpura may be
– Idiopathic (Werlhof’s disease) or
– secondary to some known etiologic factor
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Etiology of Thrombocytopenia
• Aplasia of Bone Marrow
• Displacement of Megakaryocyte in marrow
• Leukemia
• Replacement of marrow by tumour
• Destruction of marrow by irradiation, radium or
by drugs- Benzene, Aminopyrene, Arsenical
Agents
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Thrombocytopenia is characterised by

• Low platelet count
• Prolonged clot retraction & bleeding time
• Normal or slightly prolonged clotting time
• Petechiae and Hemorrhagic vesicle occur in
the palate, tonsillar pillar and buccal mucosa
• Spontaneous Bleeding in gingivae

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Gingival changes represent an
abnormal response to local irritation;
the severity of the gingival condition
is dramatically elevated by removal
of local factors
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Leukocyte (Neutrophil) Disorder
Primary neutrophil disorder
– Neutropenia
– Agranulocytosis
– Chediak Higashi Syndrome
– Lazy Leukocyte Syndrome

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Secondary Neutrophil Disorder
–Down Syndrome
–Pappilon Lefevre Syndrome
–Inflammatory Bowel Disease

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Neutropenia
• Low levels of circulating neutrophils
• Absolute neutrophil count (ANC) less
than 1500 cells/µL is considered
Neutropenia
• Neutropenia
– Genetic
– Drug Induced
– Viral infection

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• Neutropenia is serious , can lead to life
threatening infection which are difficult to control
• It may be
– chronic or cyclic
– Severe or Benign

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Agranulocytosis
• Reduction in No. of circulating
Granulocytes
• Severe Ulcerative infections of oral
mucosa,Skin, GIT and Genitourinary tracts
• Drug Idiosyncrasy MC cause of
Agranulocytosis
– Aminopyrene, Barbiturates & their deivative,
Benzene ring derivative, Sulfonamide, Gold
salts and Arsenical agents
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Agranulocytosis
• Generally occurs as Acute disease
• Chronic/Peroidic with recurring neutropenic cycles
(Cyclic Neutropenia)
• Disease onset with fever,malaise,general
weakness and sorethroat

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• Ulceration in oral cavity,oropharynx & throat is
characteristic
• Mucosa exhibits isolated necrotic patches
• Black and grey in color demarcated from adjacent
uninvolved areas
• Absence of inflammatory reaction caused by lack of
granulocytes is striking feature
10/21/13

Dr Saif Khan

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• Gingival Hemorrhage, Necrosis, Increased
Salivation and Fetid Odor
• Cyclic Neutropenia gingival changes recur with
recurrent exacerbation of disease
• Aggressive periodontitis has been seen in
cyclic neutropenia
• D/D -ANUG, Diptheria, NOMA, Acute Necrotizing
infection of tonsil
10/21/13

Dr Saif Khan

50
Chediak Higashi Syndrome
• Genetically transmitted disease
• Melanocytes, Platelets & Phagocytes affected
• Causes partial Albinism, Mild Bleeding &
Recurrent infections
• Neutrophils contains abnormal large giant
Lysosome that can fuse with phagosome but
their ability to release their content is
impaired
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Dr Saif Khan

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• Killing of ingested microrganism is delayed
• Aggressive Periodontitis

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Lazy Leukocyte syndrome
•

Defective chemotaxis response of
neutrophils
• Susceptibilty to severe microbial infections
• Abnormal inflammatory response
• Aggressive Periodontitis

10/21/13

Dr Saif Khan

54
Leukocyte Adhesion Deficiency
(LAD)
• Very rare genetic disorder
• Inherited disease-primary immunodeficiency
diagnosed at birth
• Failure to express cell- surface integrin (CD18)
which is necessary for leukocytes to adhere to the
vessel wall at site of infection
• Frequent RTI,Otitis media.primary and
permanent tooth affected early tooth loss
10/21/13

Dr Saif Khan

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Pappillon Lefevre Syndrome

• Inherited follows Autosomal Reccesive
disorder
• Parents not affected both parents must carry the
gene for the disease to manifest in offspring
• M=F predilection
• Very Rare ;1-4 cases/million

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Dr Saif Khan

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• PLS characterised by
– Hyperkeratotic skin lesion
– Severe destruction of Periodontium
– Calcification of Dura

• Cutaneous & Periodontal Changes before 4
yrs of age
• Skin lesions- Hyperkeratosis, Icthyiosis of
localised areas on palms, soles,kness and
elbows
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Dr Saif Khan

57
• Periodontal involvement
changes-

early inflammatory

bone loss

• Primary teeth are lost by 5-6 years of age
• Permanent teeth erupt normally but are lost within
because of Severe Destructive periodontal disease
• By 15 years patient is completely edentulous except
third molars
• No significant alterations have been found in peripheral
blood lymphocytes and PMNs
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Pappilon Lefevre Syndrome

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Down Syndrome

• Congenital disease caused by chromosomal
abnormality
• Trisomy 21
• Mental deficiency and growth retardation
• Prevalance of periodontal disease in Down
syndrome is 100% in patients younger than
30yrs
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Down syndrome

• Deep Periodontal pocket & substantial plaque
accumulation
• Poor PMN chemotaxis and phagocytosis
• Disease progresses rapidly
• Acute necrotizing lesions are frequent
• Increased P intermedia in children with Down
syndrome
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Influence of hematological disorder on periodontium

  • 1. Influence of Hematological disorders on the Periodontium Dr Saif Khan Assistant Professor Dept tof Periodontics & Community Dentistry Dr Z A Dental College Aligarh Muslim University, Aligarh, UP,India-202002
  • 2. Blood cells play essential role in maintenance of healthy Periodontium 10/21/13 Dr Saif Khan 2
  • 4. • Comparable oral changes occur in more than one form of blood dyscrasia • Secondary inflammatory changes produce wide range of variation in oral signs • Hemorrhagic tendencies occur when normal hemostatic mechanisms are disturbed 10/21/13 Dr Saif Khan 4
  • 6. • Abnormal Gingival Bleeding or from other areas of oral mucosa that is difficult to control may suggest some underlying Hematologic disease or Blood dyscrasia • Hemorrhagic tendencies occur when normal Hemostatic mechanisms are disturbed • Petechiaes and eechymosis of soft palate may suggest underlying bleeding disorder Dr Saif Khan 10/21/13 6
  • 7. It is essential to diagnose the specific etiology inorder to address any bleeding or immunologic disorder appropriately 10/21/13 Dr Saif Khan 7
  • 8. Leukemia Malignant neoplasia of WBC precursors Charcterised by 1.Replacement of Bone marrow with proliferating cells 2.Abnormal number & form of immature WBCs in circulating blood 3.Infiltration in liver, spleen, lymph nodes and other body sites
  • 9. Classification According to lineage of WBC, Leukemia are classified as • Lymphocytic • Myelocytic – Monocytic 10/21/13 Dr Saif Khan 9
  • 10.  According to evolution Leukemia can be – Acute – Subacute – Chronic  Acute Leukemia the primitive blast cells are released in peripheral circulation  Chronic anemia the abnormal cells are more mature with normal morphologic characteristics and function when released into the circulation 10/21/13 Dr Saif Khan 10
  • 12. All leukemic cells tend to displace normal component of Bone marrow elements with resulting in decreased production of RBC Anemia WBC Leukopenia Plateletes Thrombocytopenia 10/21/13 Dr Saif Khan 12
  • 14. Aleukemic Leukemia • Patients have normal blood counts while leukemic cells primarily reside in Bone marrow. • The peripheral blood does not contains any Leukemic or malignant cell 10/21/13 Dr Saif Khan 14
  • 15. Periodontium in Leukemia • Oral and Periodontal manifestation of Leukemia consists of – Leukemic infiltration – Bleeding or Hemorrhage – Oral Ulcerations – Infections • The expression of above signs are more common in Acute & Subacute form of leukemia than Chronic 10/21/13 Dr Saif Khan 15
  • 16. Leukemic infiltration • Leukemic cells infiltrate gingiva and less frequently alveolar bone • Gingival infiltration results in Leukemic Gingival enlargement • Highest incidence of leukemic gingival enlargement is found in patients with – Acute Monocytic Leukemia (66.7%) – Acute Myelocytic-monocytic leukemia (18.7%) – Acute Myelocytic leukemia (3.7%) 10/21/13 Dr Saif Khan 16
  • 17. • Leukemic gingival enlargement is not found in edentulous patients or in patients with chronic leukemia • Leukemic gingival enlargement consists of – Infiltration of Gingival Corium by leukemic cells – Increased gingival thickness, periodontal pocket formation, bacterial plaque accumulation and secondary inflammation 10/21/13 Dr Saif Khan 17
  • 18. Clinical features • Gingiva appears Bluish-red and Cyanotic • Rounding and Tenseness at Gingival Margin • Increase in gingival size at interdental papilla covering the crown of teeth 10/21/13 Dr Saif Khan 18
  • 21. Microscopically • Dense,diffuse infiltration of predominantly immature leukocytes in attached & marginal gingiva • Normal connective tissue component of gingiva is replaced by leukemic cells • Mitotic figures indicative of ectopic haemopoiesis may be seen • Nature of cell infiltrate depends on type of leukemia 10/21/13 Dr Saif Khan 21
  • 22. • Cellular accumulation is denser in entire reticular connective tissue layer • Pappillary layer contains comparatively few leukocytes • Blood vessels are distended and contain predominantly leukemic cells • RBCs are reduced in number 10/21/13 Dr Saif Khan 22
  • 23. • Epithelium presents with variety of changes and may be thinned or Hyperplastic • Degeneration associated with intercellular and intracellular edema and leukocyte infiltration with diminished surface keratinization 10/21/13 Dr Saif Khan 23
  • 24. • Microscopic picture of marginal gingiva differs from other gingival locations • Consists of notable inflammatory component in addition to leukemic cells • Scattered foci of plasma cells and lymphocytes with edema and degeneration are common findings • Inner layer of marginal gingiva is usually ulcerated with marginal necrosis and pseudomembrane formation 10/21/13 Dr Saif Khan 24
  • 25. • Periodontal ligament and alveolar bone may also be involved in acute and subacute leukemia • Periodontal ligament may be infiltrated with mature and immature leukocytes • Marrow of alveolar bone exhibits localised area of necrosis, thrombosis of blood vessels, infilation with mature and immature leukocytes • Replacement of fatty marrow with fibrous tissue 10/21/13 Dr Saif Khan 25
  • 26. Bleeding • Oral bleeding has been seen in 17.7% patients with Acute Leukemia and 4.4% in patient with Chronic leukemia • Gingival hemorrhage is common in leukemic patients • Bleeding gingiva can be early sign of gingivitis 10/21/13 Dr Saif Khan 26
  • 27. • Caused by Thrombocytopenia by replacement of Bone marrow cells by leukemic cells • Also by inhibition of stem cell function by leukemic cells or their products • Bleeding may be side effect of Chemotherapeutic agents used to treat leukemia 10/21/13 Dr Saif Khan 27
  • 28. Oral ulceration and Infection • Granulocytopenia (dimnished WBC count) results from the displacement of normal bone marrow cells by leukemic cells • Discrete, Punched –out ulcers penetrating deeply into submucosa and covered by a firmly attached white slough • Recurrent Herpetic oral ulcers • Atypical Oral Ulcer 10/21/13 Dr Saif Khan 28
  • 29. • Gingiva is peculiar bluish red, is sponge like and friable, and bleeds persistently on slightest provocation or even spontaneously in leukemia patients • Acute gingival necrosis • Pseudomembrane formation • NUG 10/21/13 Dr Saif Khan 29
  • 32. • Secondary oral changes superimposed on oral tissues altered by blood dyscrasia • Systemic toxic effects, loss of apetite, nausea, blood loss from persistent gingival bleeding and constant gnawing pain • Eliminating or reducing local factors (dental plaque 10/21/13 Dr Saif Khan 32
  • 33. • In chronic leukemia clinical oral changes suggesting hematologic disturbances are rare • Gingival biopsy in patient with chronic leukaemia may reveal typical gingival inflammation without any suggestion of a hematologic disturbance 10/21/13 Dr Saif Khan 33
  • 34. Anemia • Deficiency in the quantity or quality of the blood • Manifested by reduction in the number of erythrocyte and the amount of hemoglobin • Anemia results from – Blood loss – Defective blood formation – Increased RBC destruction 10/21/13 Dr Saif Khan 34
  • 35. Anemias are classified according to cellular morphology and hemoglobin content 1. Macrocytic Hyperchromic anemia (Pernicious Anemia) 2. Microcytic Hypochromic anemia (Iron deficiency anemia) 3. Sickle cell anemia 4. Normocytic Normochromic anemia (Hemolytic &Aplastic anemia) 10/21/13 Dr Saif Khan 35
  • 36. Sickle Cell Anemia • • • • Hereditary Chronic Hemolytic Anemia Exclusively in Blacks Presents with – Pallor – Jaundice – Weakness – Rheumatoid Manifestations – Leg Ulcers 10/21/13 Dr Saif Khan 36
  • 37. Oral Changes • Generalised osteoporosis of Jaw – Stepladder arrangement of trabeculae • Pallor/Yellowish discoloration • Periodontal infection may precipitate Sickle Cell Crisis 10/21/13 Dr Saif Khan 37
  • 39. Thrombocytopenia • Reduced platelet count either due to – lack of platelet production or – increased platelet destruction – Increased loss • Purpura is – purplish appearance of skin or mucous membrane that occurs as a result of decreased platelets • Thrombocytopenic purpura may be – Idiopathic (Werlhof’s disease) or – secondary to some known etiologic factor 10/21/13 Dr Saif Khan 39
  • 40. Etiology of Thrombocytopenia • Aplasia of Bone Marrow • Displacement of Megakaryocyte in marrow • Leukemia • Replacement of marrow by tumour • Destruction of marrow by irradiation, radium or by drugs- Benzene, Aminopyrene, Arsenical Agents 10/21/13 Dr Saif Khan 40
  • 41. Thrombocytopenia is characterised by • Low platelet count • Prolonged clot retraction & bleeding time • Normal or slightly prolonged clotting time • Petechiae and Hemorrhagic vesicle occur in the palate, tonsillar pillar and buccal mucosa • Spontaneous Bleeding in gingivae 10/21/13 Dr Saif Khan 41
  • 42. Gingival changes represent an abnormal response to local irritation; the severity of the gingival condition is dramatically elevated by removal of local factors 10/21/13 Dr Saif Khan 42
  • 43. Leukocyte (Neutrophil) Disorder Primary neutrophil disorder – Neutropenia – Agranulocytosis – Chediak Higashi Syndrome – Lazy Leukocyte Syndrome 10/21/13 Dr Saif Khan 43
  • 44. Secondary Neutrophil Disorder –Down Syndrome –Pappilon Lefevre Syndrome –Inflammatory Bowel Disease 10/21/13 Dr Saif Khan 44
  • 45. Neutropenia • Low levels of circulating neutrophils • Absolute neutrophil count (ANC) less than 1500 cells/µL is considered Neutropenia • Neutropenia – Genetic – Drug Induced – Viral infection 10/21/13 Dr Saif Khan 45
  • 46. • Neutropenia is serious , can lead to life threatening infection which are difficult to control • It may be – chronic or cyclic – Severe or Benign 10/21/13 Dr Saif Khan 46
  • 47. Agranulocytosis • Reduction in No. of circulating Granulocytes • Severe Ulcerative infections of oral mucosa,Skin, GIT and Genitourinary tracts • Drug Idiosyncrasy MC cause of Agranulocytosis – Aminopyrene, Barbiturates & their deivative, Benzene ring derivative, Sulfonamide, Gold salts and Arsenical agents 10/21/13 Dr Saif Khan 47
  • 48. Agranulocytosis • Generally occurs as Acute disease • Chronic/Peroidic with recurring neutropenic cycles (Cyclic Neutropenia) • Disease onset with fever,malaise,general weakness and sorethroat 10/21/13 Dr Saif Khan 48
  • 49. • Ulceration in oral cavity,oropharynx & throat is characteristic • Mucosa exhibits isolated necrotic patches • Black and grey in color demarcated from adjacent uninvolved areas • Absence of inflammatory reaction caused by lack of granulocytes is striking feature 10/21/13 Dr Saif Khan 49
  • 50. • Gingival Hemorrhage, Necrosis, Increased Salivation and Fetid Odor • Cyclic Neutropenia gingival changes recur with recurrent exacerbation of disease • Aggressive periodontitis has been seen in cyclic neutropenia • D/D -ANUG, Diptheria, NOMA, Acute Necrotizing infection of tonsil 10/21/13 Dr Saif Khan 50
  • 51. Chediak Higashi Syndrome • Genetically transmitted disease • Melanocytes, Platelets & Phagocytes affected • Causes partial Albinism, Mild Bleeding & Recurrent infections • Neutrophils contains abnormal large giant Lysosome that can fuse with phagosome but their ability to release their content is impaired 10/21/13 Dr Saif Khan 51
  • 52. • Killing of ingested microrganism is delayed • Aggressive Periodontitis 10/21/13 Dr Saif Khan 52
  • 54. Lazy Leukocyte syndrome • Defective chemotaxis response of neutrophils • Susceptibilty to severe microbial infections • Abnormal inflammatory response • Aggressive Periodontitis 10/21/13 Dr Saif Khan 54
  • 55. Leukocyte Adhesion Deficiency (LAD) • Very rare genetic disorder • Inherited disease-primary immunodeficiency diagnosed at birth • Failure to express cell- surface integrin (CD18) which is necessary for leukocytes to adhere to the vessel wall at site of infection • Frequent RTI,Otitis media.primary and permanent tooth affected early tooth loss 10/21/13 Dr Saif Khan 55
  • 56. Pappillon Lefevre Syndrome • Inherited follows Autosomal Reccesive disorder • Parents not affected both parents must carry the gene for the disease to manifest in offspring • M=F predilection • Very Rare ;1-4 cases/million 10/21/13 Dr Saif Khan 56
  • 57. • PLS characterised by – Hyperkeratotic skin lesion – Severe destruction of Periodontium – Calcification of Dura • Cutaneous & Periodontal Changes before 4 yrs of age • Skin lesions- Hyperkeratosis, Icthyiosis of localised areas on palms, soles,kness and elbows 10/21/13 Dr Saif Khan 57
  • 58. • Periodontal involvement changes- early inflammatory bone loss • Primary teeth are lost by 5-6 years of age • Permanent teeth erupt normally but are lost within because of Severe Destructive periodontal disease • By 15 years patient is completely edentulous except third molars • No significant alterations have been found in peripheral blood lymphocytes and PMNs 10/21/13 Dr Saif Khan 58
  • 60. Down Syndrome • Congenital disease caused by chromosomal abnormality • Trisomy 21 • Mental deficiency and growth retardation • Prevalance of periodontal disease in Down syndrome is 100% in patients younger than 30yrs 10/21/13 Dr Saif Khan 60
  • 62. Down syndrome • Deep Periodontal pocket & substantial plaque accumulation • Poor PMN chemotaxis and phagocytosis • Disease progresses rapidly • Acute necrotizing lesions are frequent • Increased P intermedia in children with Down syndrome 10/21/13 Dr Saif Khan 62