HEMATOLOGICAL DISORDERS

Presented ByDr. M. Shiva Shanker

Ist Year Post Graduate Student ,Dept of Periodontics, Mamata Dental College.

Contents Introduction Classification of Blood disorders Medical diagnosis Significance of bleeding disorders in

treatment of periodontal disease Management of periodontal patient with

bleeding disorders Post operative measures Conclusion References

INTRODUCTION

Blood cells play an essential role in the maintenance of a healthy periodontium. Disorders of blood cells or blood forming organs can have a profound effect on the periodontium.

Gingival and periodontal disturbances associated with blood dyscrasias must be viewed in terms of fundamental interrelationships between the oral tissues and the blood cells and blood forming organs rather than in terms of a simple association of dramatic oral changes with hematologic disease.

“Bleeding disorders are haematological conditions characterized by functional impairment of hemostatic process”

CLASSIFICATION

CELLULAR DEFECTSRED BLOOD CELL DISORDERS Anemia Erythrocytosis /

PolycythemiaWHITE BLOOD CELL DISORDERS Neutropenia Agranulocytosis LeukemiaPLATELET DISORDERS Thromobocytopenia Thromobocytosis

COAGULATION DEFECTS Haemophilia A or factor

VIII deficiency disease Haemophilia B or factor

IX deficiency Autosomal Von

Willbrands disease

Approach to the diagnosis and classification of blood diseases Imelda Bates and Barbara J. Bain

RBC Disorders

Erythrocyte disorders do not profoundly effect the periodontium. However Aplastic anemia, a bone marrow disorder characterized by marked reduction in heamopoitic tissue, has been associated with severe periodontal destruction.

Kosuke Oyaizu et al JOP 2005;76:1211-1216

Iron deficiency anemia

Due to deficiency of iron

Oral manifestations Atrophic glossitis,

mucosal pallor, and angular cheilitis.

Atrophic glossitis, flattening of the tongue papillae, resulting in a smooth and erythematous tongue may mimic migratory glossitis.

Megaloblastic anemia Due to deficiency of vitamin

B12 and/or folic acid Cracked lips, sore tongue as in

of pernicious anemia Pernicious anemia due to the lack of intrinsic

factors Oral manifestation Glossitis is most common Beefy red tongue Atrophy of tongueSchilling's test is a medical investigation used in patients with vitamin B12 deficiency.

Cobalamin deficiency is usually treated by parenteral administration of cyanocobalamin (intramuscularly or subcutaneously, 1000 μg/week for 1 month and monthly thereafter) or hydroxocobalamin in the same dose every 1–3 months intramuscularly

SICKLE CELL ANEMIA

Occurs due to gene mutation, consisting of substitution of the amino acid glutamic acid by valine 6th position on the β-haemoglobin chain.

The normal biconcave discoid shape of RBC is distorted, generally presenting a sickle like shape.

This results down to anemia and hypertophic bone marrow.

dentofacial deformities-decreased densities and coarse trabecular pattern

There was a coarse trabecular patternof “staircase” shape (present mainly in the interproximal bone because of trabeculae that formed horizontal rows),

presence of projections similar to “hair strands” due to secondary formation of bone tissue as compensation for resorption that occurred during bone marrow expansion

Gingival enlargement, firm to palpation, generaly found in the lower arch on both sides. (repeated hemorrhage episodes followed by fibrous tissue repair formation)

According to most of literature available, SCD does not appear to predispose to periodontal complications

APLASTIC ANEMIA

Bone marrow disorder Marked reduction in heamopoitic tissue Severe periodontal destruction Petechia spots, spontaneous gingival

hemorrhage frank hematomas of the mucosa, pharnyx

and gingiva Lack of inflammation development of

ulceration Blood transfusion, if there is secondary

infection antibiotics should be givenPeriodontal treatment in severe aplastic anemia, JOP

2005, 76,1211-1216.

POLYCYTHEMIA

It was first described in 1892 by Vasquez Increased in circulating RBCs due to over

production by the bone marrow, but also platelets & white blood cells.

Primary polycythemia is usually associated with a gene mutation, the cause of which is unknown.

2. Secondary polycythemia is usually associated with increased erythropoietin production variously

a. in response to low blood oxygen level b. from an erythropoietin-secreting tumour.

Signs and symptoms orofacial signs a. purplish or red areas on the i. cheeks ii. gingivaiii. lips iv. oral mucosa v. tongue b. spontaneous bleeding of the gingiva

confirmed by bone marrow sampling Treatment phlebotomy a. may increase the number of platelets b. does not reduce the size of an enlarged

liver or spleen 2. medications to reduce the risk of blood

clot.b. chemotherapy to reduce the number of red

blood cells produced by the bone marrow

A.R. Pradeep in 2011 has conducted a study to determine the association between anemia and chronic periodontitis and has stated that the chronic periodontitis may lead to anemia and provides evidence that non-surgical periodontal therapy can improve the anemic status of patients with chronic periodontitis with greater improvement in females.

THALASSEMIA The term Thalassemia was first used by

Wipple and Bradford in 1932, derived from greek word“thlassa” meaning sea and “haemia” meaning blood.

Also called as cooley’s anaemia, Mediterranean anaemia & erythroblastic anaemia

Either α or β gobulin gene may be affected. The resultant RBC have decreased haemoglobin and are thin and have shortened life span.

Types: α-thalassemia β-thalassemia thalassemia major where the patients is

homozygous thalassemia minor/trait where the patients

is heterozygous

Enlargement of the upper jaw (chipmunk

face) Migration and spacing of upper anterior

teeth Varying degrees of malocclusion (overbite,

open bite) Teeth may be discoloured, with short crowns

and roots Higher rate of dental decay Pale gums and mucosa / lining of the mouth

(due to anaemia) Sore or burning tongue (due to folate

deficiency) Painful swelling of salivary glands and dry

mouth Tooth bearing bone may have a

‘chickenwire-like’ radiological appearance

TREATMENT

Transfusion Chelation therapy Bone marrow transplantation

WBC DISODERSNeutropenia Cyclic neutropenia Benign familial neutropenia Severe familial neutropenia Chronic idiopathic neutropenia Agranulocytosis LEUKEMIADISODERS OF WBC FUNCTION Chediak-Higashi syndrome Lazy leukocytes syndrome Leukocytes adhesion defeciency Papillon- lefever syndrome

NEUTROPENIA

Characterized by decrease or absence of circulating polymorphonuclear leukocytes

Cyclic neutropenia Characterized by cyclic depletion of

polymorphonuclear leukocytes number This cyclic depression is due to mutations in

ELANE gene. Periodontal manifestation-Gingival

inflammation, Gingival ulceration, Periodontal attachment loss & bone loss

Familial neutropenia Inherited as an autosomal

dominant trait Fiery red erythematous gingiva

often hyperpastic & alveolar bone loss.

Chronic idiopathic neutropenia Persistant gingivitis- edematous

& hyperplastic

LEUKEMIA

Diffuse replacement of the bone marrow with proliferating leukemic cells.

Abnormal number and forms of immature WBCs in the circulating blood

Widespread infiltrates in the liver, spleen, lymph nodes and other body sites.

CLASSIFICATION OF LEUKEMIA

leukemia

Acute

Lymphoid Myeloid

Chronic

Lymphoid Myeloid

Factors implicated in the causation Radiation, chemical injury, genetic factors,

immune deficiency, & viral infectionPeriodontal manifestation Leukemic infiltration Bleeding Oral ulceration & infectionTreatment – chemotherapy, Radiation therapy, bone marrowTransplantation.

Classification of gingival lesions in Leukemia

Category 1 concerned with direct leukemic infilteration and

induces gingival enlargement Seen in acute monocytic & chronic lymphocytic

leukemiaCategory 2 Deals with direct drug toxicity by chemotherapeutic

agents.Category 3 Comprises the detrimental effect of graft versus host

reaction.Category4 Involves secondary effects of depression of marrow

or lymphoid tissueBarrett PA 1984

Chediak-Higashi syndrome

autosomal recessive disorder Defect – abnormalities in

cytoplasmic granules results in impaired killing of microorganism

Severe gingivitis,extensive loss of alveolar bone , premature loss of teeth deciduous and permanent

Lazy leukocyte syndrome

microbial infections, neutropenia, defective chemotactic response.

susceptible to aggressive periodontitis.Leukocyte adhesion deficiency autosomal recessive disease. LAD- combination of cell surface receptor

defect on granulocytes, lymphocytes, monocytes (Springer TA 1987).the receptors Mac-1,LAF-1 & p150,95 are involved.

Fiery red gingiva,gingival tissue bleeds readily,severe bone loss results in loss of deciduous teeth as soon as they erupt.

Papillon- lefever syndrome

Rare autosomal recessive disease

Hyperkeratosis of the palms and soles (either diffuse or localized)

Generalized rapid destruction of the periodontal attachment apparatus.

some patients suffering from PLS exhibit cellular immune defect with decreased chemotactic and phagocytic function of neutrophils and other granulocytes

Down’s syndrome

Chromosomal abnormality Mental deficiency & growth

retardation. Periodontal disease prevalence

is 100% in Down syndrome.

The main factor involved in this early periodontal destruction is thought to be the compromised immune system of people with Down’s syndrome

It results in reduced chemotaxis, diminished phagocytic ability, defective oxidative response, deficient T-cell function and abnormal bactericidal activity of polymorphonuclear leukocytes

Loesche WJ 1972.

Dental management

Advice regarding the use of fluoride toothpaste and supplements.

Advice regarding the use of antimicrobial agents such as chlorhexidine gluconate gel.

Appropriate dietary management. Use of syrup-free medications. Dental visits as often as 3 months for review

and professional support.

Platelet disorders

Thrombocytopenia Reduced platelet count

Lack of platelet production Increased loss of platelets

Petechie and hemorrhagic vesicles occur in the oral cavity

Gingiva – swollen, soft, friable Bleeding occurs spontaneously or on slight

provocation.

VASCULAR DISODERS

Hereditary vascular disorders are associated with syndromes & are characterized by blood vessel developmental abnormality.

Hereditary hemorrhagic telangiectasia Ehler-Danlos syndrome

COAGULATION DISODERS

Three disorders account for 95% of congenital disorders of blood coagulation.

These include haemophilia A or factor VIII deficiency disease

Haemophilia B or factor IX deficiency Autosomal Von Willbrands disease.Blood principles and practice of hematology, L Williams,

2003, 1103-1130.

MEDICAL DIAGNOSIS

Medical history Physical examination Laboratory evaluation.

Dental management of the classic hemophiliac with inhibitors, oral surgery oral med oral pathol 1983, 145-

8.

SIGNIFICANCE OF BLEEDING DISODERS IN TREATMENT OF PERIODONTAL

DISEASE

Illness, along with pharmacotherapy, may contribute to the tendency for excessive bleeding.

Polypharmacia and medical conditions found in an ageing population are the main reasons to reconsider treatment approaches in patient with bleeding disorders and periodontal disease.

A detailed knowledge of intraoperative and post operative hemostatic measures under challenging hemorrhagic situations is considered a priority for the dental professional.

MANAGEMENT OF PERIODONTAL PATIENT WITH BLEEDING

DISODERS

Pre-operative precautions Preoperative managements start with

thorough medical history focusing on previous bleeding history of the patient & medical condition associated with bleeding

First patient diagnosed with chronic renal failure should be managed the day after dialysis

Second, patient lacking vit k Third,the management of patients on

anticoagulant therapy

INR values Treatment

4.0 or greater No surgical treatment until INR is reduced

3.5- 4.0 Emergency minor surgical procedure only, simple extraction, incision & drainage. Avoid block anesthesia injections; use of local hemostasis

3.0-3.4 Minor surgical procedures, simple extractions,gingivoplasty avoid block anesthesia injections;use of local hemostasis

2.5-2.9 Multiple extractions, single bone impaction,quadrant periodontal surgery or SRP Avoid block anesthesia injections;use of local hemostasis

1.5-2.4 Full mouth extraction, multiple bony impactions, gingivectomy, multiple quadrant flap surgery, avoid block anesthesia injections;use of local hemostasis

Unfractionated heparin should be

interrupted 4 to 6 hrs before the surgical procedure, resumed 12 to 18 hrs after the dental procedures.

Patients taking aspirin should discontinue the medication at least 3 days, and up to 7 days, before the surgical procedure.

Preventive dental care for the patient with known bleeding disorders has to be intensive & should include regular dental visits, frequent professional tooth cleaning, oral hygiene reinforcement, fluoride supplement & mouth rinses, a low sugar diet and annual radiographic examination.

Dental care professional must be familiar of the pathology of the inherited bleeding disorders, and the dental procedure should be carried out in a facility in which the necessary equipment's and biological products are available

General medicine and surgery for dental practioners, BDJ 2003 305-10

INTRA-OPERATIVE ACTIONS

Inherited platelet disorders leading to bleeding or increased risk for bleeding are managed systemically with platelet transfusion.

Factor enhancement: Intravenous infusion of the deficient coagulation factors starts 1hr before the procedure in order to achieve a level that is 30% above the normal plasma concentration of this particular factors.

Antifibrinolysis: These are the drugs which inhibit Plasminogen activation & dissolution of clot.

Local hemostatics: Thrombin, 0.1% adrenaline solution soaked in the sterile guage, Astringents such as tannic acid

Antithrombotic agents implications in dentistry, oral surgery oral med oral pathol oral radio endod 2002: 544-551

POST-OPERATIVE MEASURES

Rinsing is prohibited on the day of the surgery and the healing site must be left undisturbed.

specific attention must be given to the tongue movements interfering with healing and food intake.

Liquids and high protein diet are strongly recommended.

The use of antifibrinolytic mouthwash is highly recommended the day after the periodontal treatment. The regimen may comprise rinsing with 10ml of 4.8-5% tranexamic acid solution four times a day for 2 minutes.

CONCLUSION

All blood cells play a role in the maintenance of a healthy periodontium.

Comparable oral changes occur in more than one form of blood dyscrasia and secondary inflammatory changes produce a wide range of variation in the oral signs.

References: Text book of periodontology, Carranza, 10th edition,

influence of systemic disorders and stress on the periodontium, Pg no: 291-299.

Periodontal medicine B C Deckar Pg no: 227-241. Pathologic basis of disease, robbins and cotran, 8th

edition Text book of physiology, Guyton. Bleeding disorders and periodontology, perio 2000,

44:211-233, 2007. Periodontal treatment consideration for cell

transplant and organ transplant patients, perio 2000, 44:82-102, 2007.

Periodontal treatment in severe aplastic anemia, JOP 2005, 76,1211-1216.

The effectiveness of a preventive regimen on the periodontal health of patients undergoing chemotherapy for leukemia and lymphoma,JCP, 1991, 18:346-347.

Periodontal implications: medically compromised patients, review, Annals of periodontology, 1,1,2006

The glycosylated hemoglobin assay for diabetes: its value to the periodontist, JOP 1989, 60:640-642.