Blood Dyscrasias

 

Dr. Pradeep Daniel Gainneos .R  PG Student  Dept. of Pedodontics

 

Introduction •

Dyscrasia (or dyskrasia) is a concept from ancient Greek medicine, medicine, meaning bad mixture.

•

The concept of of dyscrasia was developed by the Greek physician Gale Galen n (130 – 199 199 AD), who elaborated a model of health and disease as a structure of elements, qualities, humors, organs, and temperaments. temperaments.

•

Health was understood in this perspective to be a condition of harmony or  balance among these these basic components, components, called Eucrasia. Eucrasia.

•

Disease was interpreted as the disproportion of bodily fluids or four humours: phlegm, blood, and yellow and black bile. The imbalance was called Dyscrasia. Dyscrasia.

 

BLOOD Blood is a bodily fluid that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from the cells.

 

Erythrocytes or RBCs •

•

•

•

Circular, biconcave cell. Doesn’t contain a nucleus, ribosomes, or mitochondria. Contains Hb which is int Contains interwove erwoven n in the stroma. The functions of RBCs include:  –

 –

Carriage of O2 from the lungs to  peripheral tissues. tissues. Carriage of CO2.

•

Approx. 5 x 106 /cumm

•

Approx. life span- 120 days days..

 

Haemoglobin • Present in RBCs. • Required for: • O2 transport • CO2 transport • Acts as a blood buffer •

Each molecule has 2 ingredients –

•

haem hae m and globin globin..

Haemoglobin is a conjugated protein. protein.

 

Leucocytes or WBCs • Leukos= white + cytes= cells • Their fundamental job is to:  –

Providee defence Provid defence again against: st: •

 –

 –

•

Bacteria Viruses

•

Fungi

•

Parasites

Remove debris(dead cells) and foreign protein. Produce chemicals that produce inflammation.

 

Platelets •

Platelets Platele ts are o only nly aabout bout 2 20% 0% of the diameter of red blood cells.

•

The normal platelet count is 150,000-350,000 150,000-350,000 per  per microliter of  blood, but since platelets are so small, they make up just a tiny fraction of the blood volume.

•

The principal function of platelets is to prevent bleeding. bleeding.

 

Functions of blood •

Transports gases, nutrients, waste, cells and hormones throughout th roughout the body.

•

Transports O2, CO2, nutrients,

•

hormones and wastes. Regulates pH, temperature and water content of cells.

•

•

Protects against blood loss through clotting.

Protects against disease through  phagocytic white blood cells and antibodies.

 

Classification of Blood Dyscrasias Blood dyscrasias

Disorders of blood cells

Disorders of hemostatic mechanisms

Disorders of haemoglobin

Red cell

White cell

Haem

Globin

Bleeding

Coagulation

disorders

disorders

disorders

disorders

disorders

disorders

 

RED CELL DISORDER DISORDERS S

 

RED CELL DISORDER DISORDERS S •

Disorderss of RBCs numbers Disorder  –

 –

•

Anaemias Polycythemia

Disorderss in the RBC m Disorder membrane embrane  –

Hereditary Spherocytosis

 

Anaemia

 

Iron Ir on Deficiency Anaemia • Most common anaemia. • Microcytic hypochromic type of anaemia. • Women are affected 4 times more frequently than men.

CAUSES • Nutritional Deficiencies • Chronic Blood loss • Malaria

 

Systemic Syst emic features features •

•

•

 None in the early stages Cardiorespiratory  –

Dyspnoea

 –

Tachycardia

 –

Palpitations

Cutaneous  –

Pallor

 –

Koilonychi Koilo nychiaa- spoon shaped shaped nails nails

 

Oral Manifestations •

Angular stomatitis

•

Atrophic glossitis

•

Ulceration

•

Palatal pallor

 

General Management •

Administration of 300 mg ferrous sulphate orally orally,, 3 times daily for 10 days.

•

Mild gastrointestinal intolerance is the side effect.

•

Takes 10 to 20 days for the hematocri hematocritt to normalize. normalize.

•

Parenteral administration reserved for patients who do not no t respond to oral therapy. therapy.

 

Dental Considerations •

Local Anes Anesthesi thesiaa adequate adequate for pain control. control.

•

Atrophic glossitis –  glossitis – sorenes sorenesss of tongu tonguee with d depapil epapillatio lation n or colour changechange- best know known n sign of severe anaemi anaemia. a.

•

Candidiasis may be a presenting feature caused by candida albicans which may be promoted by anaemia itself.

 

Periodontal Manifestations •

CP patients with Iron deficiency anemia exhibited a higher gingival index, bleeding on probing, probing pocket depth, and increased % of sites with a clinical attachment loss (CAL) of ≥6 mm than normal CP patients.

•

In patients with significant anaemia, elective periodontal surgical procedures should be avoided, as they may cause  bleeding problems and also poor wound healing.

 

Vitamin B12 Deficiency Anaemia CAUSES o

Auto immune - Perni Pernicious cious Anaem Anaemia ia (due to atrophy atrophy of gastric mucosa).

o

Poor Intake Intake - speciall specially y in vegetar vegetarians. ians.

o

Malabsorption

o

Congenital

o

Gastrectomy Il Ilea eall di dise sease ase

o

Drugs (rare)

o

• Colchicine • Neomycin

 

General Features •

Slow to develop because liv liver er stores stores last last upto upto 3 years. years.

•

Commonly seen features are:  –

Weakness

 –

Fatigue

 –

Light-headedness

 –

Rapid heartbeat

 –

Rapid breathing

 –

Pale color to the skin

 –

In addition, neurologica neurologicall symptom symptomss particular particularly ly paresthesi paresthesiaa of the extremities may develop.

 

Oral Manifestations •

A physically normal, sore or burning tongue can be caused by early Vitamin B12 deficiency.

•

beefy red and and the tip and and margins margins Tongue may appear fiery or beefy

show papillary atrophy. •

Candidiasis can be precipitated by precipitated by anaemia.

•

Apthous stoma stomatitis titis occa occasionall sionally y present presenting ing feature. feature.

•

Marked pallor of the gingiva.

 

Dental Considerations •

Local anaesthesia satisfactory.

•

Conscious sedation- Nitrous sedation- Nitrous oxide is contraindicated. contraindicated.

•

General anaesthesia should be postponed until the low haemoglobin has been remedied. remedied.

 

Folic Acid Deficiency Anaemia Folic acid is needed by the body to 

Synthesize and breakdown amino acids



Synthesize DNA / RNA



Essential for the maturation of erythrocytes.

•

Laboratory picture is that of meg megalob aloblast lastic ic ana anaemi emia. a.

 

CAUSES: •

Mostly dietary deficiency

•

Malabsorption  –

•

•

Coeliac disease and Crohn’s disease

Increased demands  –

Pregnancy

 –

Patients under going long term renal dialysis

Drugs  –

Alcohol- interferes with metabolism and absorption absorption of folic acid.

 –

Barbiturates

 –

Phenytoin

 

General Features Folate defici deficiency ency lea leads ds to anaem anaemia ia       

Fatigue Dizziness Feeling cold Irritability

Headache Difficulty breathing Pale skin

GI symptoms such as    •

•

 Nausea, vomiting vomiting Abdominal pain Diarrhea Diarrh ea espec especially ially after after meals meals

Anorexia also is common. c ommon. In combination with the above symptoms, may lead to marked weight loss. loss.

 

Oral Manifestations •

In early deficiencies  –

•

In severe anaemia  –

•

Soreness Sorene ss of tongue without depapilla depapillation tion or colour change change

Atrophic Glossitis is the best known sign

Angular Stomatitis is also a well known sign

 

General Management •

Oral folic acid (1 mg tablet daily) is effective in reversing megaloblastic megal oblastic anaem anaemia ia caused caused b by y ffolate olate deficiency deficiency..

•

If the underlying cause of deficiency is irreversible (e.g.

intestinal disease), maintenance therapy is necessary. •

The patient should be given education emphasizing the origin of the dietary deficiency. deficiency.

 

Dental Considerations •

Local anaesthesia is satisfactory.

•

Conscious sedation can be given if the haemoglobin level is only moderately depressed and supplemental oxygen can be

given. •

General Anaesthesia should be be postponed  postponed until the low haemoglobin has been remedied.

 

Aplastic anaemia •

Pancytopenia with a non functioning bone marrow. marrow.

•

Rare disease causing leucopenia, thrombocytopenia and refractory anaemia.

CAUSES • Idiopathic • Chemicals such as benzene • Drugs • Hepatic viruses • Irradiation

 

General features Features of anaemia  –

Purpura –  Purpura  – usually usually the first manifestation

 –

Suseptibil Susep tibility ity to infection infection

 –

Suseptibili Susept ibility ty to bleeding bleeding

 

Oral manif manifestations • Ulceration • Hae Haemor morrha rhagic gic ten tenden dencie ciess Suseptibility bility to infec infections tions • Susepti • Oral Oral lichenoi lichenoid d rea reacti ctions ons

 

General Management First line treatment for aplastic anemia consists of   immunosuppressive

drugs,

typically

either

anti-lymphocyte

glob gl obul ulin in or anti anti-t -thy hymo mocy cyte te glob globul ulin in,, comb combin ined ed wi with th co cort rtic icos oste tero roid idss and an d cy cycl closp ospor orin ine. e.

 

Dental Considerations Treatment ent modifications need to take into consideration: • Treatm • Anaemia • Haemorrhagic tendencies • Susceptibility to infections • Effects of corticosteroid therapy • Hepatitis B and other viral infections

• Local anaesthesia is satisfactory satisfactory.. • Conscious sedation and general anaesthesia should be avoided. • Gingival enlargement is a complication of cyclosporine therapy.

 

Polycythemia •

Polycythem Polyc ythemia ia may be d define efined d as an abnormal increase in the erythrocyte count in the peripheral blood, blood, usually accompanied  by an increase in hemoglobin.

•

Opposite of anaemia

•

RBC count count and Hb conce concentrati ntration on both both rise. rise.

 

Polycy Pol ycythe themi miaa can be: •

Primary aka Polycythem Polycythemia ia Vera  –

•

Due to malignant changes in erythrocyte producing cells.

Secondary  –

Where there is an excess need of RBCs for survival. E.g. at high altitudes, COPD and cyanotic heart diseases. diseases .

 

Systemic Syst emic features features •

Headache

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Dizziness

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Vertigo

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Tinnitus

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Hemorrhages

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Ruddy cyanotic skin seen on the face and extremities

•

Splenomegaly is a common finding on physical examination.

 

General Management •

rythroid over productio production n and Goal of medical therapy is to reduce eerythroid to lower blood viscosity. viscosity.

•

hemato atocrit crit ris rises es above above 60%, 60%, bloo blood d is rem remove oved d usual usually ly When the hem initially,, by phlebotomy by phlebotomy every 2 to 3 days until about 500 ml initially hemato hem atocri critt is controll controlled ed..

•

•

term phlebotomy Thereafter, maintenance therapy consists of long term phlebotomy or myelo myelosuppr suppressiv essivee therap therapy y. Long term myelosupres myelosupression sion by chemo therapy therapy or irradiation irradiation is efficient.. efficient

 

Oral manif manifestations •

Facial plethora (red florid complexion) is a cardinal sign  particularly evident around the ears, nose and lips. lips.

•

Oral mucosa may have cyanotic or purplish appearance. appearance.

•

Bleeding tendencies are evident as 

Petechiae



Hemorrhages



Prolonged bleeding following extractions or periodontal surgery. surgery.

 

Periodontal Manifestations •

gingivae vae appears appears congest congested ed and edemat edematous ous and The gingi may bleed spontaneously because spontaneously because of of hyperviscosity and hypertension.

•

Gingival enlargement may be seen. seen.

 

DISORDER IN RBC MEMBRANE

 

Hereditary Spherocytosis Spherocytosis •

 – Chauffard Also known as Minkowski – Chauffard syndrome

•

Intrinsic defect Intrinsic defect in RBC RBC membr membrane ane that renders it spheroidal, less deformable and vulnerable vulnera ble to spleenic spleenic seque sequestrati stration on and

•

destruction. Mutations disrupt vertical interactions  between spectrin meshwork and intrinsic membrane proteins.

 

General features •

Signs and symptoms are highly variable

•

They may include:  –

Mild pallor

 –

Intermittent jaundice

 –

Splenomegaly.

 

Oral Manifestations •

Manifest in the mucosa as deposition of blood pigments during episodes of jaundice or in acute infections.

•

Severe dental infection may trigger an attack requiring

hospitalisation.

 

General Management •

Splenectom Splen ectomy y is frequently frequently performed performed and is invariably successful in correcting the haemolysis of erythrocytes.

•

Gall Bladder is also removed if symptomatic or diseased. diseased.

(affected in about half of the patients).

 

DISORDERS OF Hb

 

Haemoglobinopathies •

They are genetically determined disorders of haemoglobin  production.

•

Each of the haemoglobin peptide (globin) chains has a unique

amino acid sequence that can be altered as a result of DNA mutations. •

Haemog Hae moglobi lobinopa nopathie thiess are mostly seen in non –  non – caucasians. caucasians.

 

Sickling Disorders Disorders •

It is an autosomal recessive disorder .

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sickle shape shape.. Erythrocytes distort into cresen cresentt or sickle

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HbS haem haemoglobin oglobin present instead of HbA HbA..

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2 α chains are as usual but in the β chains, in position 6, valine replaces glutamic acid.

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Sickling of RBCs leads to stasis, hemolysis and microth mic rothrom rombosi bosiss of blood. blood.

 

General Features 6 main problems: Painful Crises •

 –

 –

•

Usually due to infarction brought on by infection, dehydration, hypoxia, acidosis or cold. cold. Form mainly in spleen, bones and joints, brain, kidney, kidney, lungs, retinae and skin.

Haema Hae matolo tologica gicall cri crises ses  –

 –

Caused by parvovirus by parvovirus inf infection ection.. Can be: haemolytic, aplastic or sequestration.

•

Chronic Anaemia

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Chronic hyperbilirubinaemia

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Infections- pnuemococci, Infections pnuemococci, meningococci, haem haemophilus ophilus and salmonellae

Sequestration syndromes  

Oral Manifestations •

Pallor of Pallor  of the oral mucosa.

•

•

Slow tooth development and eruption. eruption. Hypoplasia of enamel.

 

Radiographically,  –

“Step ladder” trabecular pattern

 –

Lamina Dura is more distinct

 –

Calcified pulp canals

 –

Hyperc Hyp ercem ement entosi osiss may be seen. seen.

 

General Management •

Painful and febrile crises crises of sick sickle le cell anemia are man managed aged with analgesics, sedatives, bed rest and fluids fluids..

•

When Wh en vas vasoo- occl occluso uson n de deve velo lops, ps, transfusion therapy is

advocated. •

Whole blood or packed cells are used to increase the erythrocyte count and to promote tissue oxygenation.

 

Dental Considerations •

Sickle cell anaemia with Hb less than than 11 gm/dl gm/dl presents presents a hazard hazard for general anaesthesia.

•

•

•

Local anaesthesia is the preferred mode for pain control. Conscious sedation can be used safely. safely. Preferable to avoid prilocaine which in overdose may cause Methaemoglobinaemia.. Methaemoglobinaemia

•

•

Aspirin is best avoided - may cause cause acidosis acidosis in large large doses. doses. Prophylactic antibiotics should be given and infections may be treated vigorously.. vigorously

•

Elective surgery should be carried out in the hospital and the anaemia

should be corrected corrected pre operati operatively vely with with the Hb brough broughtt upto 10gm/ 10gm/dl dl..  

Thalassaemias •

inherited disorders disorders in which which either alpha alpha These are autosomally dominant, inherited or beta globin chains are synthesized at a low rate, rate, thereby lessening the  production of Hb A.

•

Characterized by hypochromic microcytic anaemia.

•

They may be

•

 –

Severe - major thalassem thalassemia ia

 –

Mild Mil d - mino minorr thalassem thalassemia ia

May affect  –

 –

Alpha chain - alpha thalassemi thalassemiaa Beta chain chain - beta thalassemi thalassemiaa

 

Betta- Th Be Thal alas asse semi mia a •

Results from depressed production of beta chains chains..

•

Homozygous beta Homozygous  beta thalassemia (Cooley’s (Cooley’s an anae aemi miaa or thalassemia major) is characterized by:

•

 –

Increasingly severe anaemia

 –

Hepatosplenomegaly

 –

Skeletal Abnormalities

Heterozygotes for beta thalassemia may be asymptomatic.

 

Oral manif manifestations •

Marked overdevelopment of maxilla and mandible. mandible.

•

The osseous changes cause  –

Prominent cheekbones,

 –

Sunken root of the nose,

 –

Labial inclination of the maxillary incisor, incisor,

 –

Inadequate lip seal.

•

These lead to the description “chipmunk” or “rodent facies”.

•

The oral mucosa may be pale be pale due to anaemia or yellow tinged

due to jaundice.  

General Management •

•

Treatment includes includes blood  blood transfusions to temporarily control anemia anemia.. Repeated transfusions accelerate development of electromyocardial instability.

•

•

•

•

•

Iron chelating agents have been used to improve serum iron balance in these  patients. In select cases, splenectomy may be helpful. Prognosis for thalassemia thalassemia major is unfavorable and no successful therapy is available. Death usually occurs early. Thalassemia minor minor often requires requires no specefic specefic therapy unless haemoglobin

levels drop.  

Dental Considerations •

Patients undergoing repeated transfusions are at a risk of

Hepatitis B, C and HIV carriage. •

Since splenect splenectomy omy results iin n an immu immune ne defect, defect, it is prudent to cover surgical surgical procedures procedures with prophylactic prophylactic anti anti - microb microbials. ials.

•

•

Local anaesthesia is safe. Conscious sedation may be given with oxygen levels not less

than 30%. •

General anaesthesia may be complicated by enlargement of the

maxilla (difficulty in entubation).  

Periodontal Considerations •

Severe gingivitis is evident if the patient is subjected to splenectomy.

•

The colour of gingiva sometimes tends to be dark due to high

ferritin levels in the blood. •

Periodontitis, gingivitis etc. are very dangerous foci of infection in these immune impaired individuals.

 

WBC DISORDERS

 

Leukaemias •

Leuk Le ukem emias ias are are potentially lethal diseases diseases in which there is neoplastic proliferation of bone marrow WBCs.

•

•

They are classified by:  –

Clinical Clini cal course course - acute or or chronic chronic

 –

Cell of origin - lym lymphoblas phoblastt or non lymphoblast lymphoblast

Dental management of such patients is oft often en com complic plicate ated d by  bleeding tendencies and susceptibility to infection.

 

Acute Lymphoblastic Leukemia • Most common malignant disease of children. children. • Usually in childhood but can affect any age group group..

CAUSES • Ionizing radiation • Exposure to benzene

 

Clinical features • Anaemia • Lymphadenopathy • Splenomegaly • Infections • Fever • Bruising • Bleeding tendencies

 

Oral manif manifestations • Oral bleeding and petechiae are typical manifestations • Oral pallor • Gingival swelling • Mucosal or gingival ulceration • Pericoronitis • Cervical lymphadenopathy • Oral and Peri-oral Herpetic infections • Candidiasis caused by Candida albicans

 

General Management •

 prednisolone. Treatment of choice is with with prednisolone.

•

Other anti leukemic agents like anthracycline antibiotic are added if needed.

 

Dental considerations •

Preventive oral health care is essential and conservative dental treatment may be possible.

•

Surgery should be deferred except in emergencies .

•

Regional local anaesthetics contra indicated if there is severe haemorrhagic tendencies. tendencies.

•

Conscious sedation is usually possible.

•

Anaemia is a contradiction to general anaesthesia however iv sedation may be used. used.

 

Dental considerations •

Because of dangers of infection and hemorrhage, des desmo mopre pressi ssin n or  platelet or blood infusions may be needed pre operatively. operatively.

•

Antibiotic coverage should be started pre operatively and continued till the wound heals.

•

Any extraction sockets should not be packed as they predispose to infection.

•

Aspirin and other NSAIDs should not be given since they can aggravate bleeding.

 

Acute Myeloid Leukemia Most common leukemia in adults CLINICAL FEATURES • Anaemia • Lymphadenopathy • Splenomegaly • Infections • Fever • Bruising and bleeding tendencies • Night sweats • Lethargy • Weight loss l oss

 

Oral manifestations •

Oral bleeding and petechiae are typical manifestations

•

Mucosal pallor

•

Mucosal or gingival ulceration

•

Pericoronitis

•

•

Cervical lymphadenopathy

Gingival swelling - occurs du duee to abnormal abnormal resp response onse to dental  plaque

 

General Management •

Less responsive to therapy and continues to be lethal for the majority affected.

•

Remission Remissi on is the result result of intensive intensive treatm treatment ent with cytosine

arabinoside, together with anthracycline antibiotic, daunorubicin daunorub icin and some sometimes times m methotr ethotrexate. exate.

 

Dental considerations •

Preventive oral health care is essential and conservative dental treatment may be possible.

•

Surgery should be deferred except in emergencies. emergencies.

•

Antibiotic coverage should be started pre operatively and continued till the wound heals.

 

Chronic Lymphocytic Leukemia •

Primary disease of older persons.

•

The cause of CLL remains unknown.

CLINICAL FEATURES •

Many are asymptom asymptomatic. atic.

•

In others,  –

Fatigue Fever

 –

Weight loss l oss

 –

Anorexia

 –

 –

Lymphadenopathy

 –

Lymphadenopathy Haemorrhage

 

Oral manif manifestations •

Gingival bleeding

•

Oral petechiae

•

Oral ulceration

•

Herpes simplex infection may occur

•

Candidiasis is common

•

Bleeding tendencies

•

Liability to infections

 

General Management •

The chemotherapeutic agent used in the treatment of CLL is the alkyla alkylating ting agent - chlora chlorambuci mbucil. l.

•

This agent may be given with prednisone prednisone,, especially if

autoimm mmune une hemo hemolytic lytic anem anemia ia and throm thrombocy bocytopen topenia ia are autoi

•

 present. This therapy reduces nodal and spleen size and improves the quality of life.

 

Dental considerations •

Local anaesthesia is satisfactory.

•

Conscious sedation may been given.

•

General anaesthesia should be carried out in a hospital. hospital.

 

Chronic Myeloid Leukemia •

•

Most dramatic white cell disorder. Proliferation of myeloid cells in the bone the bone marrow, marrow, peripheral  blood and other tissues. tissues.

CLINICAL FEATURES  –

 –

Anaemia

 –

Weight loss lo ss Joint pains

 –

Splenomegaly

 –

Hepatomegaly

 –

Rarely lymphadenopathy

 

Oral manif manifestations • Pallor of mucosa due to anaemia • Disturbance of taste • Xerostomia • Oral hemorrhages • Swelling of salivary glands

 

General Management •

Treatment of CML is with the alk alkyla ylatin ting g age agent nt - bu busul sulfan fan or with hydroxyurea.

•

These cytotoxic drugs reduce peripheral blood counts and

spleen size, making the patient less symptomatic. symptomatic.

 

Dental considerations Treatment planning should take into consideration: • Bleeding tendencies • Liability to infection • Anaemia • Susceptibility to hepatitis B, C and HIV infection.

 

Periodontal Considerations •

Periodontal manifestations consist of leukemic infiltration, gingival bleeding bleeding..

•

Their expression is more common in acute forms of leukemia than in chronic forms.

LEUKEMIC INFIL INFILTRA TRATION TION  –

Leukemic cells can infiltrate the gingiva and less frequently the alveolar bone.

 –

Gingival infiltration results in leukemic gingival enlargem enlargement ent..

 –

Clinically,, the gingiva appears: Clinically 

Bluish red and cyanotic Rounding and tenseness of the gingival margin

Rounding and tenseness of the gingival margin 

Increase in size, mostly in the interdental papilla & partially covering the crowns of the teeth.

 

BLEEDING •

Gingival hemorrhage is a common finding in leukemic  patients.

•

It is caused by the thrombocytopenia resulting from replacement of bone marrow cells by leukemic cells and from the inhibition of normal stem cell function by leukemic cells

 

Leukemic gingival enlargement •

•

•

•

•

Most important oral sign of leukemia. True enlar enlargemen gementt occurs in acute leukemia leukemia and seldom in chronic leukemia. It may be —

diffuse or marginal

—

localized or generalized.

 Normal contours of gingiva are lost. It may appear as ―

A diffuse enlargement

―

An oversized extension of the margins

―

A discrete tumor tumor like interproximal interproximal mass

 

•

Stippling is replaced by spongy, swollen tissue that tends to  peel away from the teeth and bleed easily. easily.

•

The consistency is moderately firm, bit there is a tendency

towards friability. •

Progressive disease produces purple, produces purple, shiny and hemorrhagic gingiva.

•

Purpur Pur puric ic fea featur tures es like like petechial  petechial llesions esions and ecchymosis are

common.

 

LYMPHOMAS •

Malignant lymphomas are solid neoplastic growths of lymphoid cells that arise in reticul reticuloendot oendothelial helial and lymphoid lymphoid tissue. tissue.

•

They spread to distant lymphoid sites, such as liver, spleen and bone marrow.

•

Originate in the lymph nodes but can arise extra nodally nodally..

•

Several types of malignant lymphomas occur occur,, however those of  primary interest are  –

 –

 –

Hodgkin’s Lymphoma  Non –  Non  – Hodgkin’ Hodgkin’ss Lymphom Lymphomaa

Burkitt s Lymphoma

 

Hodgkin’s Lymphoma •

It is a neoplastic disorder originating in lymphoid tissue.

•

mass ass The disease classically begins as a painless, a painless, enlarging m that appears in the neck or mediastinum of adults.

•

Initially arises at a single site and subsequently spreads to contiguous lymphoid structures.

•

Characterized by a neoplastic neopl astic proliferation of large multi nucleated reticulum cells known as Reed Stenberg Cells. Cells.

 

General Features •

Progressive painless swelling of the cervical lymph nodes.

•

Generalized itching

•

Cyclic elevations of body temperature

•

Chills and night sweats

•

Weight loss

 

Non-Hodgkin’s Lymphoma •

•

Hetero Het erogen genous ous col collec lectio tion n of 10 different malignant lymphoid diseases. Produce numerous atypical lymphocytes that obliterate normal lymph nodal architecture.

 

General Features •

Painless lymphaden lymphadenopathy opathy –  presenting  presenting feature. feature.

•

Enlarged lymph nodes are firm, rubbery and mobile.

•

Rapid nodal growth may impair lymphatic drainage, resulting in lymphedema, pain, parasthesia and skin infiltrations.

 

Oral Manifestations •

Lymphoid tissue of Waldeyer’s Ring frequently involved site.

•

Infiltration results in non tender enlargements of tonsillar  tissue or the posterior palate.

•

The surface of these swellings may be erythematous, ulcerated or necrotic.

 

•

lymphoma, it is When palate is the sole site of primary lymphoma, When palate erativee disease of the sometimes referred to as lymphoprolif lymphoproliferativ

 palate. •

•

The enlargement is slow growing, soft, spongy and relatively  painless. lumpy,, with red surface The surface is non ulcerated and often lumpy telangiestasia.

•

As the disease progresses the colour changes from from pink  pink to  bluish purple

 

Periodontal Manifestations •

In rare rare ca cases, ses, oral Non- Hodg Hodgkins kins Lymphom ymphomaa can mimic other, more common disorders and can appear as a gingival mass.

•

Lymphomas of the gingiva are frequently frequ ently treated for months as

a pulpal or periodontal problem before the correct diagnosis is made.

 

Dental Considerations •

Preventive dental measures are most important in patients with lymphoma.

•

Generally speaking, patients in a state of remission can receive routine dental care.

•

However, patients with acute signs or symptoms of lymphoma should receive only conservative emergency dental care. care.

•

When invasive procedures procedures are required in the patient with lymphoma, bleeding time determination and consultation with oncologist are recommended.

•

Patient Patie nt undergoing chemotherapy or radiation therapy should be observed for

xerostomia or radiation caries.

 

DISORDERS OF HAEMOSTASIS

 

Haemostasis  Normal Haemostasis Haemostasis depends on on a complex interaction of the blood vessels, platelets, fibrin coagulation and deposition depos ition and fibrin fib rinolyt olytic ic pro protei teins. ns. •

Heamostas Heam ostasis is consists consists of 3 reaction reactions: s:  –

 –

Primary- vascu Primaryvascular lar phase SecondarySecon dary- plate platelet let phase phase

 –

Tertia ertiaryry- coagu coagulatio lation n phase

•

All of which occur at the same time. time.

 

Primary Haemost Haemostasis asis •

injury.. Primary haemostasis is by vasoconstriction after injury

•

This reduces the outflow of escaping blood after injury. injury.

•

collagen,, which Circulating platelets are exposed to subendothelial collagen Circulating platelets cytoplasmic mic granu granules les that stimulate causes the release of intra- cytoplas  platelet aggregation and helps to form the soft primary plug.

Abnormalities in primary haemostasis  –

Abnormal platelet number or function

 –

Abnorma Abno rmall von willebrand willebrand facto factor  r 

 

Secondary Haemostasis •

The aggregation of platelets is the second phase of haemostasis.

•

Therefore it is also known as ‘the platelet phase’. phase’.

Abnormalities in secondary haemostasis  –

Defects in the coagulation cascade cause more serious bleeding than do defects of primary haemostasis.

 –

They include bleeding into cavities (chests, joints, cranium).

 

Tertiary Haemostasis •

It is the third and final phase.

•

It is divided into 2 pathways: 

The extrinsic pathway



The intrinsic pathway

 

Bleeding Disorder Disorderss CAUSES •

Defects in haemostasis, leading to bleeding disorders can comprise:

•

 –

Platelet activation and function

 –

Anti –  Anti  – thrombin thrombin function

The more common causes include  –

Warfarin- which interferes with clotting factor factor production

 –

Vitamin K blockage

 –

Aspirin –  Aspirin  – one one tablet of which impairs platelet function for almost 1 week .

 

General Features •

•

Deep haemorrhage into muscles, joints or skin. Bleeding from and into mucosae and skin (‘bruising’)  purpura. - su sugg gges ests ts purpura.

 

Oral Manifestations •

Localized Oral Purpura

•

Blood blisters are occasionally seen, typically in older persons in the absence of obvious trauma.

•

These blood blisters are often in the palate and may sometimes  be 1 cm or more in diameter and after rupture may leave a sore area for a time.

 

PLATELET DISORDERS

 

Platelet Disorders •

Platelet disorders may be the result of  –

 –

•

Alteration Altera tion in platelet numbers numbers,, 

Decreased (thrombocytopenia (thrombocytopenia))



), Increased (thrombocythemia (thrombocythemia),

Altered platelet function (thrombocytopathia). thrombocytopathia).

Platelet disorders may be:  –

Quantitative Quantitativ e - Thrombocytopenic purpura

 –

Qualitative - Disorders of platelet function

 

Causes  –

Failed platelet production

 –

Thrombocytopenia

 –

von Willebrand’s disease.

 –

Excessive platelet Destruction

 –

Idiopathic Idiopa thic Thombocytop Thombocytopenic enic Purpur Purpura a

 –

Abnormal platelet Function

 –

Abnormal platelet Regulation

 

Thrombocytopenic Purpura •

Thrombocytopenia is a bleeding disorder characterised by a  platelet count below the normal range.

•

•

Reduced counts may be due to:  –

Failure of platelet production

 –

Disordered platelet distribution

 –

Increased platelet destruction.

It may be: 



Auto- imm immune une or Idiopa Idiopathic thic Drug induced

 

Idiopathic Thrombocytopenic Purpura •

•

immune thrombocytopenic Also known as auto – immune  purpura. It is one of the most common causes of thrombocytopenia.

•

to purpura and It is auto immune and can lead to purpura  prolonged bleeding.

 

Drug Induced Thrombocytopenic Thrombocytopenic Purpura •

Reactions of drugs or toxins t oxins resulting in a low platelet count and bleeding b leeding tendency.

•

Common offenders are:  –

Quinidine

 –

Quiunine

 –

Sulfonamides

 –

Heavy alcohol consumption.

 

General Features •

hemorrhages. emorrhages. Abrupt appearance of petechial of petechial h

•

Purpura

•

Bruises after trauma

•

Petechiae Petech iae cover extensive extensive surface surface area appearing appearing rash like, but are tender and do not itch.

•

Located chiefly on arms, lower leg, upper chest and neck .

•

In severe cases - mucosal bleeding of the GIT may occur.

 

Oral Manifestations •

Petechiae

•

Ecchymoses

•

Post operative haemorrhage.

•

Submuc Sub mucous ous purp purpur ura a may be conspic conspicous ous and some sometimes times se seen en as “blackcurrant jell y ” blood blisters. blackcurrant jelly

 

Dental Considerations •

Regional local anaesthetic block injections can be given.

•

Major surgery requires platelets more than 75x 109 /l.

•

Conscious sedation can be given.

•

General anaesthesia can be given under strict monitoring in an hospital.

•

Avoid aspirin and other NSAIDs.

 

Periodontal Considerations •

Gingiva is soft, swollen and friable.

•

Bleeding occurs on the slightest provocation and is difficult to control.

•

The severity of the gingival condition is dramatically alleviated by removal of the local factors. factors.

 

CONGENITAL COAGULATION DEFECTS •

Most important hereditary bleeding disorders include:  –

 –

von Willibrand’s disease Haemophilia Haemophi lia A and B

 

Haemophilia •

X linked disorder resulting from a deficiency in clotting factor VIII (Hemo (Hemophilia philia A or classic) classic) or fa fact ctor or IX (H (Hem emoph ophil ilia ia B or Christmas Disease) .

•

•

Affects males, females are carriers. carriers. 3 forms forms of hemophilia hemophilia are seen:  –

Severe –  Severe  –  < 1% of normal factor VIII

 –

Moderate –  Moderate  – 1 1 - 5%

 –

Mild –  Mild  – 5 5 - 25%

 

General Features •

Characterized by severe, persistent bleeding.

•

Hemo He morr rrhag hagee in into to soft tissue, muscles, organs and joints.

•

Clinically these are prominent as hematomas and

hemarthroses. •

•

Excessive bleeding after trauma. Intractable oozing or rapid blood loss one hour or more after

injury.

•

Sometimes spontaneous bleeding.

 

Oral Manifestations •

Petechiae

•

Ecchymoses

•

Spontaneous gingival bleeding can occur

•

Prolonged massive bleeding even from simple tooth exfoliation or extraction.

•

anaemia ia induce induced d pallor pallor and pseudoapht pseudoaphthae hae.. Associated with anaem

 

Dental considerations •

•

•

Local anaesthetic injections or surgery can be followed by persistent by persistent  bleeding for days. days. alone.. The hemorrhage cannot be controlled by pressure alone

•

NSAIDs.. Bleeding tendency may be aggravated by NSAIDs In all but severe haemophiliacs, non surgical dental treatment can usually be carried carried out under antifibrinolytic antifibrinolytic cover cover..

•

Extractions and dentoalveolar surgery should be carefully planned. A

•

required.. factor VIII level between 50 and 75% is required Mild hemophiliacs hemophiliacs requir requiring ing such sur surgeries geries can be managed usually without factor replacements. replacements. Desmo Desmopre press ssin in and tra tranex nexam amic ic aci acid d are  primary alternative.

•

icryll sut suture uress are most preferred Post operative suturing is desirable. Vicry

 

Periodontal considerations •

Periodontal Period ontal healt health h is of critical im importan portance ce for the hemophi hemophiliac liac for 2  primary reasons: o

Hyperemic gingival contribute contribute to spontaneous spontaneous and induced induced gingival  bleeding. o

Periodontitis is leading cause for tooth morbidity necessitating extractions.

•

In all but severe hemophiliacs, scaling can be carried out without LA LA..

•

Periodontal surgery requires LA and factor VIII replacement to a level  between 50 to 75%

 

von Willebrand’s disease •

•

•

Due to inherited deficiency in vWF. vWF. Most common inherited bleeding disorders. Affects both Affects  both males and females females..

 

General Manifestations •

Generally, bleeding m Generally, bleeding manifestations anifestations are mild.

•

Epistaxis

•

Bleeding from the mucous membrane

•

Purpura of mucous mucous membr membrane ane and skin

•

Menorrhagia in females

 

Oral Manifestations •

Gingival bleeding is the most common finding - in ab abou outt 3 30% 0% to 40% of the diseased.

•

Uncontrollable bleeding during dental procedures like gingival

manipulation and pulpectomy.

 

Dental Considerations •

Aspirin and NSAIDs should be avoided.

•

Local infilteratio infilteration n anaesthesia anaesthesia should should generally generally be used.

•

 Nerve block anaesthesia is generally contra indicated because indicated  because injections delivered into loose alveolar tissue (highly vascular regions) may produce patient’ss airway. airway.  bleeding or a dissecting hematoma that could obstruct the patient’

•

•

Conscious sedation can be given, but care must be taken not to damage the vein. General anaesthesia must be given in hospital.

•

Intubation is a possible hazard because of the risk of submucosal bleeding in the airway.

 

Periodontal Considerations •

Gingival haemorrhage is more commo common n than in haemoph haemophilia. ilia.

•

Even minor gingival manipulation can lead to uncontrollable  bleeding.

•

Cryoprecipitat Cryoprec ipitatee or other other anti fibrinolyti fibrinolyticc agents should be administered to minimize bleeding.

•

Post operative diet should be soft and semi solid to minimize trauma to the gingiva.

 

GUIDELINES FOR FOR ORAL HEALTH HEALTH CARE OF PATIENTS WITH HEMATOLOGIC DISEASE

 

Guidelines Guide lines for for Hemorrhagic Hemorrhagic Dental Dental Procedur Procedures es •

•

•

•

•

Should be avoided when patients conditions are poorly controlled. Provide prophylactic antibiotics to prevent infection when open wounds are present. Obtain pre operative CBC, DLC, platelet count, bleeding time, PT and APTT. APTT. Any abnormal values mandate a consultation. consultation . Oral surgical procedures should be done only if absolutely necessary and in a hospital setting. Single dental extractions should be performed as opposed to multiple extractions.

•

Pay meticulous attention to good technique and primary closure.

 

Guidelines for Dental Emergencies •

Pallative Pallat ive emer emergency gency treatment treatment only. only.

•

Dental infections should be treated aggressively with

•

antibiotics and drainage. Surgical procedures should be avoided until control of disease can be assessed with blood profiles.

•

 Non –   Non  – Aspiri Aspirin n or NonNon- NSAI NSAID D containing containing drugs drugs should be

•

 prescribed as post operational analgesics. If the patient comes back with bleeding

Debride the wound site and remove any clot.



Control the bleeding by packing the socket with absorbable

microfibrillar collagen or or thrombin soaked gauze. gauze.

 

Conclusion •

Recording a proper history including past medical and dental history and family history of the patient, helps the clinician in better management of  patients with bleeding disorders. disorders.

•

It not only helps in reaching to a proper diagnosis of the patient’s patient’s condition  but also in forming forming a holistic treatment plan that is beneficial for the  patient.

•

Knowledge of the signs and symptoms, both oral and systemic of these diseases enable the clinician in diagnosing cases in which the patient does not have any information about the presence of such disease

 

References 1.

Chaudhuri - Concise medical physiology, IV edition.

2.

Harsh Mohan - Essential Pathology for Dental Students, Students, 4th Edition

3.

Guyton and Hall - Textbook of o f Medical Physiology Physiology,, 11th ed

4.

Carranza’s Clinical Periodontology, Periodontology, X edition.