Rbc disorders 2/cosmetic dentistry courses

 At the end of lecture student should be able to
 Define anaemia
 Describe clinical features of thalassemia, sickle cell anaemia
and erythroblastosis fetalis
 Lab investigations of thalassemia, sickle cell anaemia and
erythroblastosis fetalis.
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 It is also known as Cooley’s Anaemia, Mediteranean
Anaemia Or Erythroblastic Anaemia
 Genetically determined disorder of Hb synthesis with
decreased production of either alpha or beta polypeptide
chains of Hb molecules, which results from markedly
decreases amounts of globin messenger RNA.

 In this condition there is diminished synthesis of alpha and
beta chain in adult haemoglobin.
 Alpha thalassemia:- deficiency synthesis alpha chain
beta thalassemia:- deficiency synthesis beta chain.

 Therefore, in beta thalassemia there is excess of alpha chain
leading to production of unstable Hb which leads to damage
of erythrocytes increasing their venerability to destruction.
 Presence of ‘Fessas bodies’ that are intercellular inclusions
which cause increase in erythrocyte hemolysis.

 Presence and absence of globin chain
 Alpha thalassemia
 Beta thalassemia
 In heterozygous
 Thalassemia minor/Thalassemia trait
 In homozygous
 Thalassemia major/Homozygous β thalassemia

 There are 2 types of thalassemia major representing alpha
thalassemia:-
 HbH disease:- mild form in which the patient lives a normal
life.
 Hb Bart’s disease:- hydrops fetalis in which infants are either
still born or die shortly after birth.

 Siblings are commonly affected
 Occurs within the first two years of life.
 Yellowish pallor of the skin
 Exhibits fever, malaise and generalized weakness.
 Splenomegaly and hepatomegaly

Face has mongoloid appearance
with prominence of cheek bones,
protrusion and flaring of
maxillary anterior teeth.
Depression of nasal bridge which
gives a characteristic rodent facies
appearance.

 Rib within a rib appearance
 Skull- Extreme thickening of
medulla and inner and outer
cortex become poorly defined.
 Hair on end or crew cut
appearance in parietal bones

 Intraoral- peculiar trabecular pattern of maxilla and mandible
 Coarsening of trabeculae and blurring and disappearance of
some- salt and pepper effect

 WBC count:- increased
 Peripheral blood smear:- Cells appear extremely pale as target
cells giving safety pin appearance
 Bone marrow smear:- cellular hyperplasia
 Serum –bilirubin level:- increased

 No treatment
 Administration of liver extract, iron tablets
 Blood transfusion
 Bone marrow transplantation

 Hereditary type
 Characterized by production of structurally abnormal HB
 HbA is genetically altered to produce substitution of valine for
glutamine at 6th position.

 More common in females
 3rd decade of life (peak)
 Weak, short breath, easily fatigued
 Pain in joints,limbs and abdomen
 Nausea and vomitting
 Systolic murmur

 Significant bone changes in the dental radiograph.
 Mild to severe generalized osteoporosis.
 No alteration in lamina dura and PDL.
 Loss of trabeculation of jaw bones with the appearance of
large, irregular marrow spaces.
 Prominent in alveolar bone.

 RBC count:- >1,000,000 cells per cubic millimetre.
 Reticulocyte count:- increased
 Blood smear:- sickle shaped RBCs
 Lactate dehydrogenase:- increased

 Congenital hemolytic anemia due to Rh incompatibility results
from the destruction of fetal blood brought about by reaction
between maternal and fetal blood factors.
 It is due to the inheritance by the fetus of a blood factor from
the father that acts as a foreign antigen to the mother.

PATHOGENESIS OF ERYTHROBLASTOSIS FETALIS

 Deposition of blood pigment in enamel and dentin in
developing teeth, giving them green, brown or blue
hue.
 Enamel hypoplasia-usually involves incisal edges of
ant. Teeth and middle portion of deciduous cuspid and
1st molar crown.
 Characteristic ring like defect occurs –Rh Hump

 RBC count:- less than 1,000,000 per cubic millimeter
 Large no. Of nucleated red cells in circulating blood

 Types of Anaemia -Thalassemia, Sickle Cell Anaemia,
Erythroblastosis Fetalis
 Clinical features of Thalassemia, Sickle Cell Anaemia,
 Lab investigations of Thalassemia, Sickle Cell Anaemia,

 Basic Pathology. Kumar, Cortan, Robbin. sixth edition.
 Shafers Oral Pathology.
 Basics of hematology. Kwathilkar.3rd edition.
 Neville Oral Pathology

Rbc disorders 2/cosmetic dentistry courses

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