Increased lysis of RBC’sNormal RBC life span is 90-120 daysIn these life span of RBC’s decreasesHaemolytic diseases result in anaemia if the bone marrow is not able to replenish adequately the premature destroyed RBC’s

CLASSIFICATION Due to intra-erythrocytic defects CONGENITAL MEMBRANE DEFECTS hereditary spherocytosis, hereditary

elliptocytosis HAEMOGLOBIN DEFECTSsickle cell anaemia, thalassaemia

other abnormal haemoglobin (Hb C, Hb D) ENZYME DEFECTS G6PD deficiency, pyruvate kinase

deficiency ACQUIRED Paroxysmal nocturnal haemoglobinuria Due to extra- erythrocytic defects Autoantibodies( autoimmune and alloimmune) Mechanical (prosthetic heart valves, microangiopathic

haemolytic anaemia) Drugs(dapsone) Infections (malaria) Inflammatory and neoplastic diseases

COMMON CLINICAL FEATURES OF HAEMOLYTIC ANAEMIASMild jaundice

Symptomns of anaemiaUrine is normal in colour on passing but turns

dark on standing due to oxidation of urobilinogen to urobilin called as “black water”

Acute back painSymptomns of cholelithiasisSplenic pain due to rapid enlargement of spleenInfections

ORAL MANIFESTATIONS

Pallor or jaundice of oral mucosaParesthesia of mucosaHyperplastic marrow spaces in maxilla,

mandible and facial bones

PALLOR OF ORAL MUCOSA

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA It is a intravascular hemolytic anaemiaVery rarely seenDue to mutation in X – linked gene, termed PIG-A

(phosphatidyl inositol glycans)Defect in stem cell is a mutation affecting myeloid

progenitor cells that normally is required for biosynthesis of glycosyl phosphatidyl inositol(GPI) needed to anchor red cell membrane.

sensitivity of RBC membrane to complement

CLINICAL FEATURES

FATIGUEPAINEOSOPHAGEAL SPASMERECTILE DYSFUNCTIONTHROMBOSIS

TREATMENTALLOGNIC BONE MARROW TRANSPLATATIONECULIZUMAB MONOCLONAL ANTIBODY

AGAINST C5 THAT INHIBITS OMPLEMENT ACTIVATION REDUCE HAEMOLYSIS

GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY ANAEMIA

INHERITED AS AN X-LINKED HEMOLYTIC ANAEMIAG-6-PD ACTS VIA HMP SHUNT TO CATALYZE THE

OXIDATION OF G-6-PD TO 6-PHOSPHOGLUCONATE GLUCOSE

ATP ADP HEXOKINASE G6PD GLUCOSE-6-PHOSPHATE 6-

PHOSPHOGLUCONATE NADP NADPH PHOSPHOENOL PYRUVATE

ATP ADP PYRUVATE OXIDIZED REDUCED KINASE GLUTATHIONE GLUTATHIONE

PYRUVATE HMP SHUNT EMBRDEN MEYERHOF PATHWAY

PRECIPITATING OR AGGRAVATING FACTORS OF HAEMOLYSIS

DRUGS

Analgesics aspirin Antimalarials primaquine , chloroquine Antibiotics sulfonamides, nalixidic acid Miscellaneous vit K, Vit C

VIRAL INFECTIONS BACTERIAL INFECTIONS DIABETIC ACIDOSIS BROAD BEAN ( VICIA FABA IN MEDITTERANEAN

VARIETY )

CLINICAL FEATURESMAJORITY OF AFFECTED PEOPLE REMAIN

CLINICALLY ASYMPTOMATIC THROUGHOUT THEIR LIVES.

MALAISEWEAKNESSABDOMINAL OR LUMBAR PAINPERIPHERAL VASCULAR COLLAPSEJAUNDICE AND DARK URINE DUE TO

INTRAVASCULAR HEMOLYSISACUTE RENAL FAILUREANAEMIA AND HAEMOGLOBINURIA ( COLA

COLORED URINE)

DIAGNOSIS BY MEASURING RBC G-6-PD ACTIVITY BY

QUANTITATIVE ASSAY

SHOULD BE DONE AFTER THE ACUTE EPISODE.

TREATMENT REMOVAL OF OFFENDING AGENT

SUPPORTIVE THERAPY FOR ANAEMIA LIKE BLOOD TRANSFUSION

TREATMENT OF INFECTION

ORAL HEALTH CONSIDERATIONS

PATIENTS SHOULD MAINTAIN EXCELLENT ORAL HYGIENE AND COMPLY WITH ROUTINE RECALL VISITS AS TO PREVENT ORAL AND PERIODONTAL INFECTION.

PROMPT AND AGGRESSIVE TREATMENT OF ORAL INFECTION ONCE DIAGNOSED IS IMPORTANT

PATIENTS SHOULD AVOID THE USE OF ASPIRIN OR OYHER DRUGS KNOWN TO TRIGGER HAEMOLYSIS.

SICKLE CELL ANAEMIAAUTOSOMAL RECESSIVE IN INHERITENCEHEMOGLOBIN GENE MUTATIONGLUTAMIC ACID VALINE AT SIXTH POSITION ON

THE β-HEMOGLOBIN CHAIN

NORMAL BICONCAVE SICKLE SHAPED DISCOID SHAPE (120 DAYS) (14 DAYS)

RESULTING IN ANAEMIA AND HYPERTROPHIC BONE MARROW

ORAL MANIFESTATIONS“STEP LADDER”TRABECULAR PATTERNENAMEL HYPOMINERALIZATION

CALCIFIED CANALS

INCREASEDOVERBITEINCREASED OVERJETPALLOR OF ORAL MUCOSA AND DELATED

ERUPTION OF TEETH

PALLOR OF ORAL MUCOSA

INCREASEDOVERJET

INCREASED OVERBITE

INVOLVEMENT OF MAXILLOFACIAL SKELETON LEADING TO RADIOOPAQUE LESIONS CORRESPOND TO BONE INFARCTS IN THE COURSE OF KNOWN VESSEL OR IN THE APICAL REGION OF TEETH

SUCH LESIONS COMBINED WITH FACIAL PAIN OR SENSORY CHANGES IN THE DISTRIBUTION OF THE INFERIOR ALVEOLAR NERVE DURING SICKLE CELL CRISIS AND ABSENCE OF DENTAL PATHOLOGY SHOULD BE CONSIDERED TO BE OF POSSIBLE VASO-OCCLUSIVE ORIGIN.

INTERRUPTION OF BLOOD SUPPLY CAN RESULT IN AN ANAESTHESIA OF INFERIOR ALVEOLAR NERVE AND PULPAL NECROSIS OF OTHERWISE SOUND PREMOLAR AND MOLAR TEETH.

RADIONUCLEOTIDE SCAN OF MANDIBLE MAY DEMONSTRATE THE POSITION AND EXTENT OF INFARCTED AREA.

OSTEOPOROSIS OF JAWS.

GARRES OSTEOMYELITIS OF MANDIBLE

GNATHOPATHY MAXILLARY EXCESS OF A 28 YEAR OLD AFRICAN AMERICAN WITH SCA

LEFT MOLAR BITEWING RADIOGRAPH RADIOGRAPHIC STEP LADDER APPEARANCE AND DENSE LAMINA DURA, RESULTING FROM HYPERPLASTIC MARROW

ORAL HEALTH CONSIDERATIONS ANTIBIOTIC PROPHYLAXIS OF CHILDREN WITH SCA FOR THE FOLLOWING

CLINICALSOLUTIONS:- DENTAL EXTRACTIONS TREATMENT UNDER GA STATUS POST SPLENECTOMY AMOXYCILLIN IS THE MOST COMMON CHOSEN ANTIBIOTIC. MAINTAINING GOOD ORAL HYGIENE ROUTINE CARE DURING NO CRISIS PERIOD AGGRESSIVE TREATMENT OF ORAL INFECTION AVOIDANCE OF USE OF ASPIRIN CAUTION WITH RESPIRATORY DEPRESSING CONCIOUS SEDATION AVOIDANCE OF LONG STRESS DENTAL VISITS USE OF NITROUS OXIDE FOR ANXIOLYSIS IS SAFE WITH MAINTAINENCE OF

ADEQUATE FLOW RATES. PREOPERATIVE TRANSFUSION OF SCA PATIENTS PRIOR TO GA MAY BE

NEEDED TO INSURE ADEQUATE LEVELS OF NORMAL HbA TO PREVENT SICKLE CELL CRISIS.

AS COMPLETE BLOOD SUPPLY IS VERY IMPORTANT DURING APPLICATION OF BOTH INTRAORAL AND EXTRAORAL FORCES SUCH PATIENTS WHO SEEK ORTHODONTIC TREATMENT REQUIRE MULTIDISCIPLINARY MANAGEMENT.

THALASSEMIAS GROUP OF GENETIC DISORDERS of Hb SYNTHESIS

CHARACTERIZED BY DISTURBANCE OF EITHER α OR β Hb CHAIN PRODUCTS.

SO CAN BE CLASSIFIED AS α THALASSEMIA β THALASSEMIA Hb

BARTS HYDROPS

FURTHER CAN BE CLASSIFIED AS α THALASSEMIA MAJOR FETALIS

Hb H

αTHALASSEMIA MINOR (TRAIT)

SIMILARLY β THALASSEMIA MAJOR (HOMOZYGOUS)

β THALASSEMIA MINOR (HETEROZYGOUS)

β THALASSEMIA IS ONE OF THE FREQUET SEEN HEMOGLOBINOPATHIES

COOLEY’S ASNAEMIA OR THALASSEMIA MAJOR IS THE SEVEREST FORM OF

β THALASSEMIA.

ORAL MANIFESTATIONSRADIOGRAPHICALY JAWS AND TEETH AMONG PEOPLE WITH

THALASSEMIA MAJOR INCLUDE:- APPEARANCE OF SPIKY SHAPED AND SHORT ROOTS TAURODONTISM

ATTENUATED LAMINA DURA ENLARGED BONE MARROW SPACES SMALL MAXILLARY SINUS

ABSENCE OF INFERIOR ALVEOLAR CANAL THIN CORTEX OF MANDIBLE

CRANIOFACIAL DEFORMITIES CLASS 2 SKELETAL BASE RELATIONSHIP WITH SHORT MANDIBLE

REDUCED POST. FACIAL HEIGHTINCREASED ANT. FACIAL PROPORTION

THINNING OF CORTICAL PLATE AT MANDIBULAR ANGLE REGION

SALT AND PEPPER APPEARANCE

O OBLITERATED SINUSES

SOME WITH SEVERE FACIAL DISFIGUREMENTS (GRADE 3 OR CHIPMUNK FACES) .

DENTAL ARCH MORPHOLOGIC CHANGES INLUDE NARROWER MAXILLA AND SMALL INCISOR WIDTHS FOR THE MAND. AND MAX. ARCHES

CONSISTENT WITH GENERAL GROWTH RETARDATION, DENTAL DEV. OF PATIENTS WITH β THALASSEMIA MAJOR WAS FOUND TO BE DELAYED BY A MEAN OF 1.61 YEARS,INCREASED WITH AGE.HIGHER FOR BOYS THAN GIRLS COMPARED WITH UNAFFECTED CHILD.

INCREASED DENTAL CARIES EXPERIENCE

• Ig A AND PHOSPHOROUS CONTENT OF SALIVA IS LESS.

FACE OFTEN DEVELOPS MONGOLOID FEATURES DUE TO PROMINENCE OF CHEEK BONES PROTRUSION OR FLARING OF MAXILLARY ANT. TEETH AND DEPRESSION OF BRIDGE OF NOSE GIVING RISE TO CHARACTERSTIC RODENT FACIES.

ORAL HEALTH CONSIDERATIONSPRIMARY CONCERN IS THE LEVEL OF ANAEMIAHEMOGLOBIN LEVELS OF 10.9 g/dl OR LESS WERE

FOUND IN 3% OF 1000 CHILDREN NEEDING MINOR DENTAL SURGERY.

PLANNED GENERAL ANAESTHESIA WERE UNDERTAKEN WITHOUT TRANSFUSION ALLOWING AUTHORS TO CONCLUDE THAT PREANAESTHETIC HEMATOLOGICAL ASSESMENT IS RARELY OF ANY SIGNIFICANCE.

MEGALOBLASTIC ANAEMIAANAEMIA DUE TO INEFFECTIVE ERYTHROPOESIS.CHARACTERIZED BY MACROCYTOSIS ASSOCIATED

WITH MEGALOBLASTIC MARROW. IT IS DUE TO VIT. B12 OR FOLATE DEFICIENCY.CAUSES OF DEFICIENCY MOST FREQUENTLY INCLUDE

FOOD-COBALAMIN MALABSORPTION SYNDROME,PERNICIOUS ANAEMIA, INSUFFICIENT DIETARY INTAKE, AND MALABSORPTION.

PERNICIOUS ANAEMIA IS AN AUTOIMMUNE DISEASE RESULTING FROM AUTOANTIBODIES DIRECTED AGAINST INTRINSIC FACTOR AND GASTRIC PARIETAL CELLS.

ORAL MANIFESTATIONSBURNING SENSATIONS IN TONGUE, LIPS, BUCCAL

MUCOSA AND OTHER MUCOSAL SITES.TONGUE AND MUCOSA MAY BE SMOOTH OR

PATCHY AREAS OF ERYTHEMA.DYSPHAGIA AND TASTE ALTERATIONS HAVE BEEN

REPORTED.ATROPHY OF PAPILLAE OF TONGUE RESULTING

IN HUNTER’S OR MOELLER’S GLOSSITIS

TONGUE LOOKS BALD AND BEEFY RED IN COLOR

ERYTHEMA OF LIPS

GLOSSODYNIA