Oral Medicine: Red Cell Disorders
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Questions and Answers

What is the average lifespan of a red blood cell?

120 days

Which of the following is a characteristic of polycythemia?

  • Increased red blood cell count (correct)
  • Decreased red blood cell count
  • Increased white blood cell count
  • Decreased white blood cell count

What is the most common type of anemia, especially in women of childbearing age?

  • Sickle cell anemia
  • Iron deficiency anemia (correct)
  • Aplastic anemia
  • Pernicious anemia

Folate deficiency anemia is characterized by neurological manifestation.

<p>False (B)</p> Signup and view all the answers

What are two genetic conditions that can cause a form of anemia called Hemoglobinopathies?

<p>Sickle cell disease and Thalassemia</p> Signup and view all the answers

What is the name of the genetic disorder that leads to a characteristic change in shape of red blood cells, due to an amino acid substitution?

<p>Sickle Cell Disease</p> Signup and view all the answers

What is the term for the genetic condition where there is a deficiency in the synthesis of alpha or beta globin chains?

<p>Thalassemia</p> Signup and view all the answers

What is a common oral manifestation in sickle cell disease?

<p>Delayed eruption and enamel hypoplasia (B)</p> Signup and view all the answers

In Sickle Cell Disease, a radiograph of the skull might show the appearance of "hair on end".

<p>True (A)</p> Signup and view all the answers

Which of the following is NOT a common feature of aplastic anemia?

<p>Increased blood viscosity (A)</p> Signup and view all the answers

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Flashcards

Polycythemia

Increase in the number of red blood cells (RBCs) in the peripheral blood, usually with higher hemoglobin and hematocrit.

Absolute erythrocytosis

A true increase in red blood cell mass.

Relative erythrocytosis

Red blood cell mass is normal, but plasma volume is reduced, making the red blood cell count appear higher.

Relative polycythemia cause: Loss of intravascular fluid

Occurs when there is a loss of intravascular fluid, such as during rapid diuresis for congestive heart failure.

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Primary polycythemia (Polycythemia Rubra Vera)

Unknown etiology, common in elderly patients, primarily men.

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Secondary polycythemia (Erythrocytosis)

Caused by a variety of factors including low oxygen levels, tumors, and certain medications.

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Anemia

A reduction in the oxygen-carrying capacity of the blood, usually due to a decrease in red blood cells or hemoglobin.

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Normocytic anemia

Red blood cells are normal in size.

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Microcytic anemia

Red blood cells are smaller than normal.

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Macrocytic anemia

Red blood cells are larger than normal.

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Normochromic anemia

Hemoglobin concentration is normal.

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Hypochromic anemia

Hemoglobin concentration is low.

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Iron deficiency anemia

Most common type of anemia, especially in women of childbearing age.

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Etiology of iron deficiency anemia

Inadequate iron intake, consumption of tea with meals (tannic acid interferes with iron absorption), impaired iron absorption (gastric issues), chronic blood loss (heavy menstruation), and increased iron requirements (pregnancy, growth).

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Koilonychia

Spoon-shaped and brittle nails.

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Oral manifestations of iron deficiency anemia

A pale oral mucosa, angular cheilitis, glossitis (sore, atrophic tongue), sore mouth, and slow wound healing.

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Plummer-Vinson syndrome (Patterson-Kelley syndrome)

Smooth, red, and painful tongue.

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Plummer-Vinson syndrome: Mucosal atrophy

Atrophy of the mucosa in the mouth, pharynx, and upper esophagus.

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Plummer-Vinson syndrome: Dysphagia

Difficulty swallowing due to muscular degeneration and stenosis of the esophageal mucosa.

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Pernicious anemia (Addisonian anemia)

Progressive destruction of the gastric fundic glands leading to atrophic gastritis, loss of intrinsic factor, and vitamin B12 malabsorption.

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Etiology of pernicious anemia

Autoantibodies against the parietal cells of the stomach, leading to a deficiency in intrinsic factor secretion by parietal cells.

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Vitamin B12 function

Vitamin B12 is essential for red blood cell maturation and nerve tissue metabolism.

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Clinical features of pernicious anemia

Symmetrical numbness of the extremities, muscular weakness, constipation or diarrhea, vague epigastric pain, and increased risk of stomach cancer.

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Oral manifestations of pernicious anemia

Glossitis, glossodynia (painful tongue), sore atrophic tongue, pallor of the oral mucosa, aphthous-like ulceration (especially on the tongue), intolerance to dentures due to mucosal changes, and disturbed taste sensation.

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Folate deficiency anemia

Macrocytic normochromic anemia caused by a deficiency of folic acid.

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Causes of folate deficiency anemia

Poor intake, malabsorption (e.g., inflammatory bowel disease), increased demand (e.g., infancy and pregnancy), and drugs (alcohol, barbiturates, phenytoin).

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Aplastic anemia

Rare bone marrow aplasia, causing refractory normocytic normochromic anemia, leukopenia (low white blood cell count), and thrombocytopenia (low platelet count).

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Etiology of aplastic anemia

Congenital (e.g., Fanconi's anemia), acquired (e.g., immune suppression), drugs (cytotoxic and noncytotoxic), ionizing radiation, and viral infection (e.g., hepatitis and measles).

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Oral manifestations of aplastic anemia

Pallor of the oral mucosa, minimal but persistent gingival bleeding, petechiae and ecchymosis (bruising), and oral ulcers.

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Hemoglobinopathies

Defects in the globin portion of the hemoglobin molecule, making red blood cells more susceptible to hemolysis (breakdown).

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Sickle cell disease

Hereditary disorder of hemolytic anemia, where glutamic acid on the beta chain of hemoglobin is replaced by valine.

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Sickle cell trait (HbAS)

Heterozygous cases where 60% of hemoglobin is HbSS, the remainder is normal hemoglobin. Patients are usually asymptomatic, but can experience sickle cell crisis under reduced oxygen tension.

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Thalassemia

A group of hereditary diseases characterized by deficient synthesis of either alpha or beta chains of hemoglobin.

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Cooley's anemia

Most severe type of thalassemia, characterized by slow growth, ashen gray skin color, recurrent bacterial infections, severe anemia, hepatosplenomegaly, and skeletal growth retardation.

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Study Notes

Oral Medicine, Diagnosis & Periodontology I

  • Students are responsible for all material presented in lectures and sections, even if not in handouts.
  • Textbook: Burket's Oral Medicine, 12th edition.

Red Cell Disorders

  • Dr. Amira Abdelwhab
  • RBC lifespan: 120 days.
  • RBC count: 4.5-5 million/cubic mm (males); >females.
  • Hemoglobin: 13-16 gm/dL (males); 13-14 gm/dL (females).
  • Adult hemoglobin (Hb A): α2β2
  • Fetal hemoglobin (Hb F): α2γ2

Classification of Red Blood Cell (RBC) Disorders

  • Polycythemia
    • Absolute erythrocytosis: true increase in red cell mass
    • Relative erythrocytosis: red cell mass normal, but plasma volume reduced -Causes: intravascular fluid loss, rapid diuresis (secondary to congestive heart failure treatment) -Hemoglobin rarely rises >25% -No significant oral changes
  • Anemia
    • Normocytic, normochromic
    • Microcytic, hypochromic (iron deficiency anemia)
    • Macrocytic, normochromic (pernicious anemia, folate deficiency)

Primary (Idiopathic) Polycythemia

  • Etiology: unknown
  • Age and gender: elderly (males > females).
  • Characterized by:
    • High number of RBCs (up to 18 million/mm3).
    • High hemoglobin (18-24 g/dL).
    • Elevated white blood cell (WBC) count.
    • Elevated platelet count with dysfunction.
    • High blood viscosity.
    • Elevated serum iron and ferritin levels.

Secondary Polycythemia

  • Etiology: varied causes (e.g., heart defects causing 2ry polycythemia, brain tumor)

Clinical Features of Polycythemia

  • Symptoms/signs due to high blood volume and viscosity.
  • Thrombosis as a complication of elevated blood viscosity
  • Bone marrow overactivity

Extra-Oral Features of Polycythemia

  • Purplish red face, extremities;
  • Distended dark neck veins;
  • Headaches;
  • Bone Pain;
  • Splenomegaly (enlarged spleen).

Oral Features of Polycythemia

  • Purplish-red oral mucosa
  • Varicosities in the ventral aspect of the tongue
  • Bleeding gums
  • Petechiae and ecchymoses

Dental Management of Polycythemia

  • Particular attention to hemostasis during dental procedures.

Anemia

  • Defined as a reduction in oxygen-carrying capacity of the blood, typically due to reduced numbers of red blood cells or decreased hemoglobin.
  • Types of anemia:
    • Normocytic/normochromic anemia
    • Microcytic/hypochromic anemia (e.g., iron deficiency anemia)
    • Macrocytic/normochromic anemia (e.g., pernicious anemia, folate deficiency)

Iron Deficiency Anemia

  • Etiology:
    • Inadequate iron intake
    • Impaired iron absorption (e.g., gastrectomy)
    • Chronic blood loss (e.g., heavy menstruation)
    • Increased iron requirements (e.g., pregnancy, growing children, or consumption of tea with meals)
  • Clinical features:
    • Iron depletion
      • Sparse hair
      • Brittle nails
      • Spoon-shaped (koilonychia) nails
    • Anemia and tissue hypoxia
      • Fatigue
      • Headaches
      • Palpitations
      • Dyspnea on exertion
      • Pallor of skin, nail beds, and palpebral conjunctivae
      • Tachycardia
      • Cardiac murmurs
  • Oral features:
    • Pallor of oral mucosa
    • Angular cheilitis
    • Glossitis
    • Sore mouth and atrophic oral mucosa
    • Slow wound healing

Plummer-Vinson Syndrome

  • Smooth, red, painful tongue
  • Atrophy of the mucosa of the mouth, pharynx; upper esophagus
  • Angular cheilitis and oral ulcerations
  • Dysphagia (difficulty swallowing)
  • Mucosal atrophy due to iron deficiency

Pernicious Anemia

  • An autoimmune disease where the stomach's parietal cells are progressively destroyed.
  • Etiology:
    • Circulating autoantibodies.
    • Defective intrinsic factor secretion.
    • Defective vitamin B12 absorption.
  • Defective RBC maturation
  • Clinical features:
    • Insidious onset
    • Anemia
    • Severe pallor
    • Jaundice
    • Neurologic symptoms
    • Gastrointestinal symptoms (e.g., constipation, diarrhea, epigastric pain, increased risk of stomach cancer
  • Oral features:
    • Glossitis, glossodynia (painful tongue), and sore atrophic tongue
    • Pallor of the entire oral mucous membrane
    • Aphthous-like ulcerations, particularly on the tongue
  • Diagnosis:
    • Detailed history
    • Clinical examination
    • Laboratory investigation: decreased RBC count, reduced hemoglobin, increased MCV, MCH, reduced serum vitamin B12 testing
  • Treatment:
    • Lifelong vitamin B12 injections

Folate Deficiency Anemia

  • Macrocytic normochromic anemia due to folate deficiency
  • Body reserve of folate in the liver can be depleted in less than 4 months.
  • Causes:
    • Poor intake
    • Malabsorption
      • Inflammatory bowel disease
    • Increased demand
      • Infancy
      • Pregnancy
    • Drugs that interfere with folate absorption or metabolism
      • Alcohol
      • Barbiturates
      • Phenytoin
  • Oral features(s) as with other types of anemia
  • Diagnosis: Normal Schilling test, low serum folate level, normal serum vitamin B12 level.
  • Treatment: Folic acid supplements (5 mg/day).

Aplastic Anemia

  • Rare bone marrow aplasia
  • Refractory normocytic normochromic anemia
  • Leukopenia and thrombocytopenia (pancytopenia).
  • Etiology: -Congenital causes (like Fanconi's anemia)
    • Acquired causes (immune suppression of bone marrow stem cells by T suppressor cells; drugs, e.g., cytotoxics like methotrexate, and noncytotoxics like chloramphenicol; radiation exposure; viral infections)
  • Clinical features: Anemia, thrombocytopenia, and neutropenia are the principal clinical features. Frequent infections, bleeding, and fatigue are common. Oral manifestations include pallor of the oral mucosa, minimal or persistent gingival bleeding, petechiae and ecchymoses, and oral ulcers.
  • Diagnosis: Detailed history and clinical examination; laboratory investigation for pancytopenia; normal MCV, MCH, and MCHC
  • Treatment: Addressing the cause, bone marrow transplant is vital, sometimes.

Hemoglobinopathies

  • Defects in the globin portion of the hemoglobin molecule; making RBCs with abnormal hemoglobin susceptible to hemolysis.
    • Sickle cell disease
    • Thalassemias
      • major
      • Minor

Sickle Cell Disease

  • Hereditary disorder of hemolytic anemia
  • Glutamic acid on beta chain is replaced by valine
  • Clinical features:
    • Painful crisis - abdominal and bone pain due to blood vessel occlusion by sickled RBCs
    • Aplastic crisis - cessation of RBC production.
    • Splenic sequestration crisis - early childhood, spleen becomes enlarged as sickle cells entrap there, potentially fatal.

Sickle Cell Trait

  • Heterozygous, mild form
  • No significant clinical manifestations
  • Increased risk in situations of hypoxia (e.g., general anesthesia)

Thalassemia

  • Inherited group of anemias with deficient synthesis of either alpha or beta globin chains.
  • Clinical features:
    • Slow growth & development of children.
    • Ashen gray skin color
    • Recurrent bacterial infections (increased iron availability).
    • Severe anemia (features of).
    • Hepatosplenomegaly
    • Skeletal growth retardation
    • Iron overload (due to repeated blood transfusions)
  • Oral manifestation(s): Pale oral mucosa, Yelllowish tinge, delayed eruption and enamel hypoplasia, maxillary overgrowth, painful swelling of parotid glands caused by iron deposition, painful tongue, dentin and enamel with excessive quantity of iron (5 times normal).
  • Radiologic features: similar to sickle cell anemia.

Diagnosis:

  • Specific hematological finding(s) like reduced RBCs, reduced hemoglobin, reduced MCH, MCHC, MCV, elevated Fetal Hemoglobin
  • Identifying factors like case history; clinical examination & laboratory investigation testing (including Schilling Test, Antibody for parietal cells, gastric intrinsic factors, Serum Folate).

Treatment:

  • Eliminating causative factors/ addressing the underlying issue (like infection)
  • Symptomatic treatment; supportive therapy like IV Fluids, oxygen, antibiotic and narcotic analgesics
  • Medications like Folic acid (during pregnancy; severe hemolysis).
  • Blood transfusions (as in aplastic crisis; reduced Hb levels)
  • Bone marrow transplant

Dental implications of the above conditions:

  • Avoid extensive soft tissue surgery due to slower healing time.
  • Avoid general anesthesia if Hb is low.
  • Treat any infections promptly.

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Description

This quiz covers material from the Oral Medicine, Diagnosis & Periodontology I course, focusing specifically on red cell disorders. Students will review key concepts related to red blood cell lifespan, classification, and abnormalities such as polycythemia and anemia, referencing Burket's Oral Medicine. Prepare to test your knowledge on the specifics of RBC disorders and their implications.

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