Blood & Lymph Disorders-Pathology

Front 1

What are the 4 RBC diseases?

Back 1

1. Iron Deficiency Anemia
2. Pernicious Anemia
3. Sickle Cell Anemia
4. Thalassemia

Front 2

1. How/Why does Anemia occur?
2. What are causes of Anemia?

Back 2

1. Reduction in RBC oxygen transport or Reduction in RBC: size, number, Hb content

2. Excessive loss of blood, Hb production deficiencies, and Nutritional deficiencies

Front 3

What are the causes of Iron Deficiency Anemias?

Back 3

1. Heavy menstrual bleeding
2. GI Bleeding
3. Poor Iron absorption
4. Increased iron demand (pregnancy)

Front 4

What are the clinical features of Iron Deficiency Anemia?

Back 4

May be asymptomatic
May cause weakness/fatigue

Oral manifestations:
-angular cheilitis
-atrophic glossitis
-Mucosal pallor

Front 5

What is Plummer-Vinson Syndrome? (Iron deficiency anemia)

Back 5

Rare condition in middle aged
Common in Scandinavian women
Long standing Iron deficiency
**Predisposition to oral and esophageal SCC

Front 6

Histopathology of Iron Deficiency Anemia?

Back 6

Based on Lab tests:
1. RBC count
2. Hb levels
3. Hematocrit

Front 7

What is the treatment for Iron Anemia?

Back 7

Dietary Fe supplements (Ferrous sulfate)
Improvement w/in several months

Front 8

Name the clinical features of Pernicious Anemia.

Back 8

Fatigue, weakness, headache, shortness of breath.
Burning painful tongue
Numbness/tingling/parasthesia in extremities
Atrophic, pale, erythematous oral mucosa

Front 9

What percent of people w/ pernicious anemia have loss of filiform papilla?

Back 9

50%

Front 10

1. What is the Diagnosis for Pernicious Anemia?

2. What is the treatment?

Back 10

1. Large (macrocytic), dark (hyperchromic) RBC
and Low Vit B12 Levels

2. IM injections of B12
*Oral conditions improve w/ treatment

Front 11

What type of genetic trait is Sickle Cell anemia and what populations are most commonly affected?

Back 11

1. Autosomal recessive
2. African-Americans and Mediterranean decent

Front 12

Describe the pathogenesis of Sickle Cell Anemia

Back 12

1. Substitution of valine for glutamate at position 6
2. On deoxygenation Hb molecules aggregate
3. Cannot pass through the capillaries; more rbc destroyed

Front 13

What are symptoms of SC Anemia?

Back 13

Weakness, fatigue, malaise, shortness of breath, delayed growth, infection.

Front 14

What are the radiographic features of Homozygous SC anemia?

Back 14

"step-ladder" trabeculation of posterior mandible

"Hair-on-end" appearance of calvarium

Front 15

What is the treatment for SCA?

Back 15

Supportive care: Blood transfusions and O2 administration

Front 16

What type of genetic trait is Sickle Cell anemia and what populations are most commonly affected?

Back 16

1. Autosomal recessive
2. African-Americans and Mediterranean decent

Front 17

Describe the pathogenesis of Sickle Cell Anemia

Back 17

1. Substitution of valine for glutamic acid at position 6
2. On deoxygenation Hb molecules aggregate
3. Cannot pass through the capillaries; more rbc destroyed

Front 18

What are symptoms of SC Anemia?

Back 18

Weakness, fatigue, malaise, shortness of breath, delayed growth, infection.

Front 19

What are the radiographic features of Homozygous SC anemia?

Back 19

"step-ladder" trabeculation of posterior mandible

"Hair-on-end" appearance of calvarium

Front 20

What is the treatment for SCA?

Back 20

Supportive care: Blood transfusions and O2 administration

Front 21

T/F=Thalassemia is autosomal dominant?

Back 21

F= Autosomal recessive

affects the synthesis of alpha and beta-globin Hb chains

Front 22

List the 3 homozygous forms of Thalassemia and the 2 heterozygous forms.

Back 22

Homo:
1.Thalassemia Major
2.Cooley's Anemia
3.Mediterranian Fever

Hetero:
1.Thalassemia Minor
2. Midly symptomatic

Front 23

What are the clinical features of Thalassemia Major

Back 23

-occurs in early life
-Jaundice, fever, weakness
-Maxillary enlargement

Front 24

What is the maxillary enlargement in Thalassemia from?

Back 24

Bone marrow hyperplasia
Protrusion of anterior teeth
"Chipmunk facies"

Front 25

What do the radiographic films look like for Thalassemia major-
SKULL/ DNETAL

Back 25

SKULL: hiar on end appearance
Dental: honeycombed appearance of trabeculae

Front 26

What is used to diagnose Thalassemia Major?

Back 26

Hb electophoresis
-Both forms have hypochromic and microcytic RBCs

Front 27

What is the treatment for Thalassemia Major?

Back 27

Blood transfusions
*Multiple transfusions can cause hemochromatosis or increased Fe deposition

Front 28

What is Fetal Erythroblastosis?

Back 28

Hemolytic anemia of the newborn
-From transplacental Ab attack on fetus antigens

Front 29

What causes Fetal Erythroblastosis?

Back 29

Rh- mother having an Rh+ baby only after subsequent pregnancies with 1st Rh+ baby
*HEMOLYSIS of fetal RBC= jaundice

Front 30

What can be clinical signs in a fetus with fetal erythroblastosis?

Back 30

-Cyanosis and jaundice
-Increased bilirubin from RBC hemolysis is toxic to brain-->kernicterus
-Teeth yellow/green
-Normal color only after trt increased bilirubin

Front 31

Diagnosis of FE?

What is the test of the mother?

Back 31

-decreased Hb, hematocrit, or RBC count
-Increased unconjugated bilirubin

Test: Coombs test for mothers antibody detection

Front 32

What is the treatment for FE?

Back 32

Infant transfusions
-Can be prevented if proper immunoprophylaxis for Rh- mothers.
-Anti-Rh IG (w/in 72 hrs of delivery)

Front 33

What is Leukopenia?

Back 33

A decrease in WBC count <

Causes an increase in bacterial/fungal infections

Front 34

List the 2 subtypes of Leukopenia?

Back 34

-Agranulocytosis
- Cyclic neutropenia

Front 35

What is Agranulocytosis?

Back 35

Decrease in the # of granulocytes (usually neutrophils)

-May be idiopathic
-Drug related (cancer treatment, chemo, etc.)

Front 36

What 2 syndromes can produce Agranulocytosis?

Back 36

1. Congenital Agranulocytosis
2. Myelodysplastic syndrome

Front 37

What are the clinical features of Agranulocytosis?

Back 37

-bacterial infections common
-fever, malaise, chills, bone pain
-punched-out necrotic ulcerations of oral mucosa

Front 38

What is the diagnosis and treatment for Agranulocytosis?

Back 38

1. WBC count

2. Bone marrow cytokine stimulation, Antibiotic therapy, and cessation of chemo may restore WBC count

Front 39

What is cyclic neutropenia?

Back 39

Periodic decrease in neutrophils
-rare
-cyclic decline occurs 21-30 day cycle

Front 40

What is the WBC count to be considered cyclic neutropenia?

Back 40

< 3,000/mm2 for 4-5 days

Front 41

What are the clinical signs for cyclic neutropenia?

Back 41

-Fever, malaise, sore throat, URI
-Oral ulcerations: lips, tongue, buccal mucosa, gingiva
-Severe PDD in children

Front 42

Diagnosis and Treatment for cyclic neutropenia?

Back 42

-Periodic WBC counts every 6-8 wks

-Antibiotic therapy for infections
-cytokine therapy to increase neutrophil production

Front 43

Name the types of Neutrophil Function Disorders

Back 43

1. Chronic Granulomatous Disease of childhood

Front 44

What are the general characteristics of Neutrophil function disorders?

Back 44

-Normal numbers, but decreased function
-Can affect chemotaxis, phagocytosis, enzymes
-Can be caused by certain drugs (corticosteroids)

Front 45

What are some general features of Chronic Granulomatous Disease of childhood?

Back 45

-Genetic component
-X linked recessive
-Affects males

Front 46

What type of neutrophil defect is involved with chronic granulomatous disease of childhood?

Back 46

Decreased production of H2O2 and supra oxide
-due to NADPH oxidase defect

Front 47

CGD of childhood commonly affects females more than males? T or F

Back 47

False- affects males more often

Front 48

Clinical features of CGD of chilhood include:

Back 48

-occurs in childhood/early teens
-Recurrent bac/fungal infections common (Strep/staph, pseudomonas/candida)
-Osteomyelitis, abscess, dermatitis
-Granulomas of lungs/lymphnodes

Front 49

What are the oral manifestations of Chronic Granulomatous Disease of Childhood?

Back 49

-Candida infections
-Ulcers
-Gingivitis
-Early Periodontal disease

Front 50

Treatment of CGD of childhood?

Diagnosis?

Back 50

-Antibiotic/Anti-fungal therapy
-Interferon/bone marrow transplants

-decreased Nitroblue tetrazolium (NBT) by granulocytes

Front 51

Leukocytosis is an increase in the # of WBC, usually from immature to mature? T/F

Back 51

True
May be due to an infectious disease or malignancy

Front 52

What are the general features of Infectious mononucleosis?

Back 52

-transmitted by healthy carries by close saliva contact
-Infects epithelial cells and B-lymphocytes
-B lymphocytes activate T lymphocytes

Front 53

What is the virus that causes infectious mono?

Back 53

Ebstein-barr virus
-Member of the herpes group

Front 54

What are signs/symptoms of mono?

Back 54

-Fever, lymphadenopathy, sore throat, rash, splenomegaly later, malaise, and fatigue

-Occurs in young adults
- 7-10 day incubation period
- Oral ANUG has been reported

Front 55

Diagnosis/Treatment of Mono?

Back 55

- WBC count 15-20,000/mm3
-smear= atypical lymphocytes
-mono spot test= EBV antibodies
-Infection may resolve in 4wks

Treat: bed rest, antipyretics, and analgesics