ANEMIA

Definition reduction in the level of haemoglobin

levels, which is usually accompanied by a decrease in the number of erythrocytes ( RBC’s), resulting in lowering the O2 capacity of the blood.

Normal Hb concentrations in blood for adults:

Males : 13.5 g/dl Females : 11.5 g /dl

The mean corpuscular volume, or "mean cell volume" (MCV), is a measure of the average red blood cell volume that is reported as part of a standard complete blood count

The hematocrit (Ht or HCT) or packed cell volume (PCV) or erythrocyte volume fraction (EVF) is the percentage of blood volume that is occupied by red blood cells

Causes: 1) chronic blood loss and consequent iron

deficiency, usually in women from heavy menstruation

2) folate and vitamin B12 deficiency ( the 2nd common cause ).

Classification according to RBC size:

In patients with anemia, it is the MCV measurement that allows classification as either a

microcytic anemia (MCV below normal range),normocytic anemia (MCV within normal range) macrocytic anemia (MCV above normal range).

The normal MCV is 78-99 fL, with smaller cells (<78 fL) described as microcytic and larger cells (>99 fL) as macrocytic.

Microcytic anemia : most common usually due to iron deficiency , occasionally to thalassaemia or chronic disease.

Macrocytic anemia: usually caused by vitamin B12 or folate deficiency ( sometimes that is caused by pregnancy, chronic haemolysis, malignancy or some drugs, liver disease , myxoedema or aplastic anemia

Normocytic anemia : chronic disease like leukaemia , chronic inflammatory disease , renal failure , infection, malignancy and sickle cell anemia

Clinical features: early: asymptomatic.

pallor of the oral mucosa and conjunctiva,

tiredness, dyspnoea, tachycardia, murmurs and palpitation.

General management 1) Clinical and laboratory investigation.( MCV, Hb, blood film )

2) Hematinics. ( iron or folic acid)

3) Erythropoietin. (used for anemia in chronic renal failure or cytotoxic therapy)

4) Blood transfusion. ( used only when absolutely nesseccary Hb under 7.0 g/dl)

Dental aspects - some anemias cause oral lesions such as ulcers,

glossitis or angular stomatitis.

-L.A - conscious sedation: only if there is supplemental O2

- Nitrous oxide : contraindicated in B12 def.

- G.A : it is vital to ensure full oxygenation.

- elective operation: Hg more than 10 g/dl. - Preoperatively : raise the hemoglobin level if

necessary by transfusion.

In details

1) Iron deficiency anemia. 2) Vit B12 deficiency. 3) folate deficiency.

Iron deficiency anemia General aspect: Most common cause of anemia in the west, as a result of

nutritional deficiency or chronic blood loss.

Developing countries: malaria and chronic blood loss. Main cause of microcytic anemia.

Clinical features: 1) impaired excersie capacity. 2) koilonychia: "spoon nails." It refers to abnormally

thin nails (usually of the hand) which have lost their convexity, becoming flat or even concave in shape.

General management

Iron salt supplement such as ferrous sulphate, or ferrous gluconate .

Oral iron may need to be given for 3 months or more after the hemoglobin has reached normal levels, to replenish marrow iron stores.

Parenteral iron : intramuscular.

Dental aspects: L.A satisfactory for pain management

Conscious sedation: if full oxygenation is possible.

At early stages: sore tongue can develop if hemoglobin falls beneath the normal value.

Severe anemia: atrophic glossitis, soreness of the tongue with depapillation or colour change.

Dental aspectscont.

Candidosis can be aggravated or precipitated by anemia

Treatment with iron appears to improve the response to antifungal treatment.

Angular stomatitis(affects only minority).

Aphthous stomatitis is sometimes associated with iron deficiency anemia.

Vitamin B12 deficiency General aspects: B12 needed for A.A, DNA/RNA, new cells.

Found in the diet in the meat.

Deficiency of vit B12 is usually due to defect in intrinsic

factor( as a result of pernicious anemia or gastrectomy)

Nitrous oxide is contraindicated if given more than 12 hours, interfere with metabolism of Vit B12.

General aspectscont.

Pernicous anemia: autoantibodies against gastric parietal cells, or to the intrinsic factor.

Clinical features: Deficiency develops slowly(liver stores last up to 3

years) Neurological symptoms: paraesthesiae in the

extremities. Treatment : I.M hydroxycobalamin

Dental aspects: L.A is satisfactory Conscious sedation can be given if the Hb level is

moderately depressed and supplemental oxygen can be given.

Nitrous oxide is theoretically contraindicated.

G.A : if Hb is not low.

Early B12 def: normal or sore or burning tongue. Red sore patches may form on the tongue,(pattern or

red patches without depapillation).

Dental aspectscont.

Candidosis can be aggravated or precipitated by anemia and may be the presenting feature.

Angular stomatitis (uncommon)

Aphthous stomatitis is occasionally the presenting feature.

Folate deficiency Folic acid is needed A.A/ ,DNA/RNA ,new cells

Folic acid is found in fresh leafy and other vegetables.

No body stores of folic acid.

Most folic acid def. is caused by dietary def.

Clinical features: Folate def. in adluts leads to anemia.

In pregnancy folate def. leads to neural tube defects or cleft lip-palate in the fetus.

Effects of folate def. is very similiar to B12 def.

Red cell folate are more reliable than serum folate.

Treatment: folic acid

Dental aspects: L.A, conscious sedation, GA: the same as pernicous

anemia.

No contraindication of nitrous oxide.

Atrophic glossitis is the best known effect of severe anemia.

Angular stomatitis (affects only a minority).

HAEMOLYTIC ANAEMIAS

Inherited abnormality of Hb function

Defected structure or function of erythrocytes

( spherocytosis, G6DP def.)

Damage of erythrocytes ( autoimmune, drug induced, infective)

Clinical features :

Billirubin over production ( Jaundice) Spleen enlargement Increased RBC turnover to cause in the

end an increase in folic acid demand, and macrocytic changes occur

Treatment:

Folic acid Transfusion ( risk of iron overload)

Dental aspects :

LA : safest method of pain control Conscious sedation: given with

supplemental oxygen if nesseccary GA: Contraindecated

* main concern : sickle cell anemia

Sickle cell anemia ( autosomal)

The hemoglobin that is present is HbS

Gross distortion in erythrocyte shape

Might cause vascular occlusion since erythrocytes are stiff

Sickle cell anemia Trait: • Heterozygous • X10 more common than Sickle Cell Disease• Asymptomatic

Sickle cell crises is due to low oxygen tension Sickle cell anemia is most common in

africans, afro-caribbeans and middle eastern Early mortality rates are high

Sickle cell disorder: ( homozygous)

Clinical Features : 1. Painful crises ( mainly due to infarctions

especially in the spleen , bone, kidney , brain, lungs, joints.. Etc)

2. Haematological crises 3. Chronic hyperbillrubenemia 4. Infections ( mostly pneumococci,

meningiococci, heamophilus, salmonella) 5. Chronic anemia6. Sequestration syndromes: chest( gas change

impairment ) , blood, girdle ( bowels)

Management

Family history should be taken Take notice if Hb is less than 9 g/dl Blood film to follow the shape of RBC’s Electrophoresis: 40% Hbs , 60% HbA

Treatment Folic acid should be taken regularly Monitoring of cells Comprehensive care programme Blood transfusion ( usually avoided hep. C,

and HIV)

* * Main cause of death: thrombosis , infection

Prevent trauma, infection, hypoxia, acidosis, dehydration ( to avoid a crises)

Dental aspect GA : investigate all sickling disorders

especially when the Hb is less than 11 then there’s a hazard

Sickle Cell Trait: GA : few problems, full oxygenation should

be given

Dental Aspects cont.Sickle Cell Disease: LA : preferred to stop the pain Avoid prilocain> methaminoglobinemia Avoid asprin > acidosis Use acetaminophens, codiene Conscious sedation : used safely with relative

analgesia Elective surgery : only when hemolysis is

minimal Prophylactic antibiotics: penicillin V ,

clindamycine , should be given before surgery

Oral manifestations Painful infarcts in the jaws (radio opacities )

might be mistaken for osteomyelitis or toothache

Stepladder trabeculae pattern ( in xrays) Skeletal maturation is delayed Crises might be predisposing to osteomyelitis Hypercementosis might be seen Bone marrow hyperplasia leads to enlarged

heamopoietic maxilla with excessive over jet Enamel hypo mineralization and calcified pulp

canals

THALASSEMIA

Thalassemia Autosomal dominant inherited disorder

Alpha and beta globin chains will be produced slowly thus low HbA

Erythrocytes fragility will be due to the excess in the production of the not affected chains

Characterized by microcytic anemia

Found in Mediterranean , middle eastern

Thalassemia is divided into : Sever ( major, homozygous) Mild ( minor , heterozygous)

Alpha thalassemia: ( alpha chain) Beta thalassemia ( beta chain )

Alpha thalassemia

Alpha chain deficient Found in asians No compensatory mechanisms Lethal in utero or infancy due to inability

of carrying Oxygen

Beta thalassemia Mediterranean anemia Beta chains Homozygous : cooley anemia ( major

type) Severe anemia, failure to thrive,

hepatosplenomegaly, skeletal abnormalities

Heterozygous : might be asymptomatic

Clinical features HOMOZYGOUS Chronic anemia Marrow hyperplasia Skeletal defomaties Splenomegaly Cirrhosis Gallstones Iron overload : will damage the heart and

cause death

HETEROZYGOUS: asymptomatic Or mild hypochromic anemia

General managment Severe microcytic anemia ( confirmed) Basophilic stippling of erythrocytes Normal or raised serum iron and ferritin Normal total iron-binding capacity Great increase in fetal Hb Some increase in HbA2

treatment Blood transfusions Folic acid and chelating agents Ascorbic acid Hydroxycarbamide Splenectomy : if hyper- splenism happens

Dental aspects LA: safe Conscious sedation: if oxygen level not

less than 30% GA: complicated by the enlargement of

maxilla in intubation, and it is contraindicated in the presence of severe anemia and cardiomyopathy

Oral manifestations Enlarged maxilla ( chipmunk facies) Hair-on-end appearance due to expantion of

the diploe Less common: parotid swelling , xerostomia

due to iron deposition, sore or burning tongue due to folate deficiency

Pneumatization of the sinuses may be delayed

Spacing of the teeth and forward drift of the maxillary incisors ( ortho might be recommended)

Skicle Cell trait with haemoglobinopathy

SCT with thalassaemia are not usually as ill as those with isolated sickle cell disease

They are at the same level of risk from general anaesthesia as are those with sickle cell disease

Patients with other combined defects should be managed in the same way as those with sickle trait alone

Erythrocyte membrane defects

General and clinical aspects: Hereditary spherocytosis is the main form of

congenital haemolytic anemia in Caucasians Autosomal dominant Characterized by :• Heamolytic anemia • Jaundice• Splenomegaly• Gallstones• Heamochormatosis • Skin infections

General management Splenectomy Folic acid treatment

Dental Aspects: LA is safe, conscious sedation and GA may

be given at optimal oxygenation

Erythrocytic metabolic defects G6PD deficiency : most common

Acquired hemolytic anemias Caused by: Trauma , toxins , complement mediated

lysis and malaria

Aplastic anemia

Pancytopenia with a non-functioning bone marrow , causes leucopenia , thrombocytopenia and refractory anemia

Clinical features Same as normochromic, normocytic or

macrocytic together. Susceptibility to infection and bleeding Purpura is the first manifestation

General management Removal of the cause : • stop chloramphenicol• Bone marrow transplant • Steriods• Blood transfusion

Prognosis is poor and 50% of the patients die within 6 months , usually form haemorrhage or infection.

Dental aspects Take consideration of: Anemia Haemorrhagic tendency Hep. B , and other viral infections

Ulcers Susceptibility to infections Oral lichenoid lesions Sjogren like syndrome Gingival swelling

Fanconi’s anemia Rare autosomal recessive syndrome where 90%

develop bone marrow failure (the inability to produce blood cells) by age 40.

characterized: by: Skeletal defects Hyper pigmentation Pancytopenia

Susceptible to head and neck carcinoma at early age as a result of a genetic defect in a cluster of proteins responsible for DNA repair

Pure red cell aplasia Congenital or acquired , refers to a type of

anemia affecting the precursors to red blood cells but not to white blood cells. the bone marrow ceases to produce red blood cells.

Associated with: thymoma ( tumor of epi. Of thymus) Sometimes chronic mucocutaneous candidosis

Those patients need regular blood transfusions

Anemia caused by bone marrow infiltration

Infiltration by abnormal cells cause normocytic anemia and often leucopenia or thrombocytopenia

Dental care may be complicated by susceptibility to infections, haemorrhage or underlying disease

Anaemia associated with systemic disease

Including: Chronic inflammation ( infection or rheumatoid

arthritis ) Neoplasms ( leukemia) Liver disease

Very rarely: Hypothyroidism Hypopituitarism Hypoadernocorticism Uraemia HIV infection

THE ENDLana Obeidat

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