Hematologic Disorders

Question 1

What are things that look yellow clinically?

Answer 1

• Fat
• Lymphoid Tissue
• Keratin
• Purulent Exudate
• Calcification
• Neural Tissue

Question 2

Plasminogen Deficiency

Answer 2

• No plasminogen=No plasmin=Clots keep growing
• Fibrin Builds up
• Seen in 1st decade of life
• LINEOUS CONJUNCTIVITIS
  
  • thick yellow plaques in conjunctiva mucosa of upper eyelid
• Oral:
  
  • Mostly Gingiva:
    
    • patchy, ulcerative plaques
    • irregular borders
    • multiple quads
  • Laryngeal Muosa
    
    • vocal cords→raspy, hoarse voice

Question 3

Hemophhilia: Types

Answer 3

• Hemophhilia A
  
  • aka classic hemophilia
  • Factor 8 deficiency
  • F=carrier, Males are affected
  • most common hemophilia
  • Hemarthrosis
    
    • pseudo tumor of hemophilia
      
      • deep hemorrhage of Knee after normal activities
  • Dental Care:
    
    • administer clotting factor before surgery
• Hemophilia B
  
  • aka Christmas disease
  • Factor 9 deficiency
• Von Willebrand Disease:
  
  • most common inherited bleeding disorder
  • Von Willebrand Factor Deficiency (plasma glycoprotein)
    
    • aids in adhesion of platelets at site
    • binds to factor 8→acts as transport module
  • Abnormal platelets

Question 4

Anemia

Answer 4

• Decrease in RBCs or Hemoglobin concentration
• results from:
  
  • decreased production of erythrocytes
  • increased destruction or loss of erythrocytes
• Immunosuppressed to a degree
• Treat the underlying cause
  
  • anemia=condition not diagnosis
• Hemoglobin > 8mmHg (normal)

Question 5

Anemia Clinical Features

Answer 5

• Tired
• Light headed
• Headache
• Papitations w/exercise
• Leg cramps

Question 6

Anemia: Oral Manifestations

Answer 6

• Pale oral mucosa
• loss papillae fro tongue
• angular chilitis
• oral infections
• Aphthae (RAUs)
• Burning or sore tongue
• Ulcerative stomatitis
• Gingival Hypertrophy
• Gingival Bleeding

Question 7

Sickle Cell Anemia

Answer 7

• Most severe Genetic Disorder of hemoglobin synthesis
  
  • mutation: Substitute T→ A
  • codes for Valine instead of glutamic acid
  • Hb→ rigid and curved (sickle)

Question 8

Sickle Cell Trait

Answer 8

• only 1 gene affected (1 allele mutation)
• resistance to malaria

Question 9

Sickle Cell Disease

Answer 9

• both alleles mutated
• sickle cells block capillaries

Question 10

Sickle Cell Crisis

Answer 10

• Pain and fever
  
  • caused by hypoxia, infection
• Infarction of bones, lungs, abdomen→extreme pain
  
  • Acute chest syndrome
    
    • lung involved due to embolism or pneumonia
• Susceptible to S. pneumoniae
  
  • most common cause of death In children

Question 11

Sickle Cell Anemia: Oral Manifestations

Answer 11

• “Hair on end” radiographic appearance of skull bones
• Coarse “Step Ladder” bone trabeculae
  
  • bone marrow hyperplasia w/increase hematopoeisi
• Osteomyelitis of mandible
• Mandibular bone infarction
• Prolonged Paresthesia of IAN
• plural necrosis
• Plummer-Vinson Syndrome
  
  • web like growth in throat→difficult to swallow

Question 12

Sickle Cell Anemia: Dental Treatment

Answer 12

• only modify procedure if anemia is severe
• Avoid elective procedure
• Avoid LA w/epinephrine stringer than 1:100,000
  
  • avoid septocaine and prilocaine
• Avoid:
  
  • Barbituates
  • Narcotics
  • NSAIDs
  • Aspirin
• Adequate oxygen during nitrous oxide
• Pulse Ox monitoring
• supportive therapy to avoid bleeding complications
• Antibiotics→Low WBC

Question 13

Aplastic Anemia

Answer 13

• FATAL
  
  • unable to produce alll the types of blood cells
  • most common in immune-mediated cytotoxic T-lymphocytes that targets marrow cells
• Associated with Dyskeratosi Congenita

Question 14

Aplastic Anemia: Clinical Features

Answer 14

• RBC deficiency
• Thrombocytopenia
  
  • bruise easily
• Neutropenia, Leukopenia, or granulocytopenia
  
  • predisposition to bacterial or fungal infections

Question 15

Aplastic Anemia: Oral Manifestations

Answer 15

• Related to thrombocyotpenia
  
  • pale oral mucosa
  • Gingival Hemorrhage
  • oral mucosa petechia and ecchymoses
  • oral ulcerations (RAUs)
  • Gingival hyperplasia

Question 16

Neutropenia:

Answer 16

• Neutrophil Count< 1500mm3
• infection of the oral mucosa=initial sign of disease
  
  • especially AA and Middle Eastern
  • Benign Ethnic Neutropenia
• Infancy: associated with
  
  • Dyskeratosis congenita
• Adults:
  
  • Drugss
  • Metabolism
  • Bacterial/Viral Infections
  • Vit B12 deficiency
  • SLE (lupus)

Question 17

Neutropenia: Clinical Features

Answer 17

• increased bacterial infections

Question 18

Neutropenia: Oral Manifestations

Answer 18

• Premature perio w/early exfoliation of deciduous teeth
• ulcerations of gingival tissue

Question 19

Lymphoma vs Leukemia

Answer 19

• Lymphoma
  
  • starts in lymph nodes or other parts of lymphatic system (arm pits, groin, neck)
• Leukemia:
  
  • starts in the blood and bone morrow

Question 20

Leukemia

Answer 20

• Malignancies of hematopoietic stem cell derivation
• Can be due to environmental exposure (5%)
  
  • pesticide, benzene, ionization radiation, virus

Question 21

Classification of Leukemia

Answer 21

• Classified by: Histopathology and clinic behavior
  
  • Acute or chronic
    
    • acute
      
      • aggressive
      • fatal
    • chronic
      
      • idiopathic
  • Myeloid or Lymphoblastic/Lympocytic
    
    • Myeloid
      
      • differentiate along severe pathways
• Acute Myeloid:
  
  • broad age range
  • Myelodysplasia syndromes, early precursor
  • 2nd most common in children
• Chronic Myeloid
  
  • Peak incidence: 3rd/4th decade
  • Philidelphia chromosome: BCR-ABL fusion gene 22;9
• Acute Lymphoblastic
  
  • most common in children (2-4 y.o.)
• Chronic Lymphoblastic
  
  • most common type
  • older adults

Question 22

Leukemia: Oral Manifestations

Answer 22

• Myeloid Sarcoma
  
  • granulocytic sarcoma
  • extramedulary myeloid tumor
  • chloroma

Question 23

Langerhand Cell Histiocytosis

Answer 23

• BRAF Gene Mutation
• >50% of patients are < 15 y.o.
• Classification:
  
  • single organ involvement: (Bone or skin)
    
    • unifocal
    • multifocal
  • Multi-organ involvement
    
    • no organ dysfunction
    • organ dysfunction
      
      • low risk vs high risk
• Oral Manifestations:
  
  • Pushed-out lesion on x-ray
  • Teeth floating in air
  • mostly mandible

Question 24

Hodgkin Lymphoma