Professional Documents
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HEMATOLOGICAL DISORDERS
Thalassemias
ERYTHROCYTOSIS : POLYCYTHEMIA VERA
IDA is defined as a reduction in total body iron to an extent that iron stores are
fully exhausted and some degree of tissue iron deficiency is present. It may occur
as a consequence of low dietary intake, impaired absorption, or excessive iron
loss.
ACI also known as anemia of chronic diseases, ACI is observed in autoimmune
diseases, acute and chronic infection, malignancies and chronic kidney disease. In
ACI, the utilization of existing iron stores is impaired, resulting in iron
sequestration.
Clinical and Oral Manifestations:
Pallor of conjunctivae, lips, and oral mucosa, brittle nails, palmer creases.
Other signs and symptoms of anemia includes angular chelitis, pale oral mucosa,
PLUMMER-VINSON SYNDROME
Also known as Paterson-Kelly syndrome
It is a rare syndrome characterized by the classic triad of dysphagia, iron deficiency
anemia and upper esophageal webs
Oral manifestations includes glossitis, glossopyrosis, glossodynia and angular
cheilitis
Barium swallow test is done to diagnose oesophageal webs(thin projections on ant.
wall)
Oral Health Cosiderations:
Routine dental care should be deferred in those patients whose Hb is <11 g/ dL or
those manifest sign and symptoms such as shortness of breath, abnormal heart rate
or oxygen saturation less than 91% until their health status improves
Dentists should be aware that anemia places a patient at increased risk of ischemic
heart disease.
MACROCYTOSIS:
B12 AND FOLATE DEFICIENCY ANEMIA
Due to decreased intake, impaired
absorption and increased requirements
Clinical and Oral Manifestations :
Fatigue, decreased mental concentrations,
weakness
Includes beefy red tongue with smooth or
patchy area of erythema, soreness or a
burning sensation affecting the tongue,
lips, buccal mucosa and other mucosal
sites. Paresthesia and taste alterations
have been reported
Hunter's glossitis showing fiery red
appearance in megaloblastic anemia
HEMOLYSIS: PAROXYSMAL NOCTURNAL
HEMOGLOBINURIA
PNH is a rare acquired condition characterized by bone marrow failure, hemolytic
anemia, thrombosis, and smooth muscle dystonia.
Clinical manifestations includes fatigue, pain, esophageal spasm, erectile
dysfunction and platelet activation
Glucose-6-Phosphate Dehydrogenase
Deficiency
G6PD deficiency is inherited as an X-linked
hemolytic anemia caused by mutations in the
G6PD gene and more than 400 mutational
variants have been defined.
Clinical Manifestations:
The vast majority of individuals with G6PD
deficiency remain clinically asymptomatic
throughout their lives.
Characteristic signs and symptoms of fatigue,
flank pain, anemia, and jaundice are evident.
Oral Health Considerations:
Patients with G6PD deficiency can tolerate the delivery of all the necessary
dental care. Medications known to induce a hemolytic crisis should be promptly
managed. G6PD patients should maintain excellent oral hygiene and comply with
routine recall visits so as to prevent oral and periodontal infection.
HEMOGLOBINOPATHIES:
Sickle Cell Disease
It is a chronic hemolytic blood disorder characterized by abnormal Hb
which under low oxygen tension, results in sickling of cell. It is a
severe genetic disorder of Hb synthesis. It is autosomal dominant.
Clinical and Oral Manifestations: Acute complications of SCD
include acute chest syndrome, aplastic crisis, fever/sepsis,
osteomyelitis, pain, priapism, splenic sequestration and stroke.
Chronic conditions of SCD include asthma, avascular necrosis, gall
bladder disease, hepatic dysfunction, leg ulcers, nephropathy,
pulmonary hypertension and silent infarcts.
Oral findings includes mucosal pallor, delayed eruption, discolored and
depapillated tongue and increased periodontitis.
Oral Health Considerations:
SCD is not associated with increased periodontal disease, mandible osteomyelitis
precipitating a sickle cell crisis is reported
Amoxicillin was the most common antibiotic,& perceived the higher risk of
infectious complications was highest for extractions, followed by restorative
treatment and tooth polishing
for patients deemed low risk, simple surgical procedures may be accomplished in
the out patient setting.
Pain management may be chalanging and usually consists of anti inflammatory
agents, opioid/ nonopioid analgesics and proper hydration. For patients deemed
at moderate or high risk, dental therapy should be rendered in a hospital setting
where appropriate medical support is readily available.
Thalassemias
The thalassemias are a group of inherited autosomal recessive disorders of Hb
synthesis characterized by a disturbance of either alpha or beta Hb chain
production.
ALPHA THALASSEMIA : There is reduction or absence of alpha chain synthesis. Alpha
chains of Hb are required not only for HbA but also foe HbF, which is the main
haemoglobin type in fetal life and result in intrauterine death of fetus.
BETA THALASSEMIA : There is reduction or absence of beta chains. Hemolysis not
primarily due to lack of beta globin chains but it is because of the free alpha chains
which form insoluble aggregates that precipitate within the RBCs and cause
damage to the cell membranes.
Clinical and Oral Manifestations
Patients with α- thalassemia trait are asymptomatic.
Non-Hodgkin’s Lymphoma
Burkitt’s Lymphoma
Multiple myeloma