5 Hematologic Diseases Flashcards

1
Q
  • responsible for recognition and processing of foreign antigens (viruses, fungi, bacteria)
  • protective function
A

lymphoid tissue

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2
Q

During response to antigenic challenges, how do lymph cells respond?

A

lymph cells proliferate –> results in enlargement of the lymphoid tissue (hyperplasia)

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3
Q

Sites of lymphoid tissue:

A
  • lymph nodes
  • lymphoid tissue of Waldeyer’s ring
  • aggregates of lymphoid tissue normally scattered throughout the oral cavity: oropharynx, soft palate, lateral tongue, floor of mouth
  • head and neck region
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4
Q

Where can lymphoid hyperplasia occur?

A

any site of lymphoid tissue

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5
Q

Intraoral indications of lymphoid hyperplasia:

  • description
  • size
  • color
  • locations (3)
A
  • discrete, nontender, submucosa swellings
  • < 1 cm in diameter
  • normal, dark pink, creamy yellow-orange hue
  • posterior lateral tongue, floor of mouth, wall of pharynx
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6
Q

reasons for enlarged lymph nodes: acute vs chronic infection

A

Acute- enlarged, tender, relatively soft, freely moveable

Chronic- enlarged, rubbery firm, nontender, freely movable, history of a previous inflammatory illness, should be a lack of progressive enlargement

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7
Q

differentials (2) for multiple persistently enlarged nontender lymph nodes

A

lymphoma

HIV infection

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8
Q

peak of tonsillar size

A

peaks early during the second decade of life (usually more prominent in younger people)

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9
Q

symmetric vs asymmetric LARGE tonsils

A

As long as the large tonsils are symmetrical and asymptomatic, we do nothing!

Tonsillar asymmetry is a potentially serious sign that should be evaluated further to rule out the presence of a malignancy.

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10
Q

decrease in the volume of RBCs (hematocrit) or in the concentration of hemoglobin

A

anemia

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11
Q

often a sign of underlying disease

A

anemia

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12
Q

Symptoms related to decreased O2 carry capacity due to anemia:

A
  • tired
  • headache
  • lightheaded
  • pallor of mucosa
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13
Q

genetic disorder of hemoglobin synthesis–> hemoglobinopathy

A

sickle cell anemia

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14
Q

genetic cause of sickle cell anemia

A

thymine substituted for adenine in DNA –> altered codon results in valine instead of glutamic acid in the beta-globin chain of hemoglobin

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15
Q
  • deoxygenated hemoglobin molecule is prone to molecular aggregation and polymerization
  • RBCs undergo deformation and take on a rigid sickle shape
A

sickle cell anemia

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16
Q

sickle cell trait vs disease

A

Sickle cell trait = one allele is affected (45-50% of hemoglobin is normal, no significant clinical manifestations)

Sickle cell disease = both alleles affected (1:350-400 black people in the US)

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17
Q

1:350-400 black people in the US

A

sickle cell disease

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18
Q

tissue/organ affected by sickle cell anemia

A

any tissue or organ

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19
Q

Clinical findings of sickle cell anemia? How many patients have severe involvement?

A
  • highly variable clinical findings

- 1/3 of patients have severe involvement

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20
Q

patients with sickle cell anemia are more prone to these infections

A

S. pneumoniae (thought that susceptibility develops following splenic destruction from repeated infarctions)

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21
Q

delayed growth and development from sickle cell anemia

A

impaired kidney function and ocular abnormalities due to repeated occlusive episodes in small vessels

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22
Q

when the sickling of erythrocytes becomes severe

A

sickle cell crisis

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23
Q

Causes (5) of sickle cell crisis:

A
  • most have unknown cause
  • hypoxia
  • infection
  • hypothermia
  • dehydration
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24
Q

A sickle cell crisis causes extreme pain from ______ and ______ of affected tissue. What are the most common affected locations?

A

ischemia and infarction / long bones, lungs, and abdomen most affected

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25
Q

How long do episodes of sickle cell crisis last?

A

3-10 days

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26
Q

tx for sickle cell crisis

A
  • supportive care, fluids, rest, analgesics

- avoid exposure to cold, dehydration, and strenuous exercise to avoid sickle cell crisis

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27
Q

oral manifestation: hair on end appearance of skull bones

A

sickle cell anemia

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28
Q

oral manifestation: reduced trabecular pattern of the mandible due to increased hematopoiesis in marrow

A

sickle cell anemia

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29
Q

oral manifestation: asymptomatic pulpal necrosis

A

sickle cell anemia

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30
Q

oral manifestation: increased prevalence of osteomyelitis

A

sickle cell anemia

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31
Q

tx for sickle cell anemia

A
  • continuous prophylactic penicillin therapy until age 5 to prevent S. pneumoniae infection
  • pneumococcal vaccination
  • hydroxyurea –> increases fetal form of hemoglobin
  • avoid exposure to cold, dehydration and strenuous exercise to avoid sickle cell crisis
  • genetic counseling
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32
Q

tx: continuous prophylactic penicillin therapu until age 5 to prevent S. pneumoniae infection

A

sickle cell anemia

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33
Q

tx: pneumococcal vaccination

A

sickle cell anemia

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34
Q

tx: hydroxyurea –> increases fetal form of hemoglobin

A

sickle cell anemia

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35
Q

tx: genetic counseling

A

sickle cell anemia

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36
Q

disorders of hemoglobin synthesis due to reduced synthesis of either the alpha-globin or beta-globin chains of the hemoglobin molecule

A

thalassemias

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37
Q

among the most common inherited human conditions

A

thalassemias

38
Q

Degree of clinical severity of thalassemia varies depending on? How many mutations have been documented for beta-thalassemia alone?

A

depends on genetic alteration and if it is heterozygous or homozygous / 200 different mutations

39
Q

heterozygous vs homozygous state of thalassemia

A

Heterozygous- adequate amount of normal hemoglobin can be made, affected patient experiences few signs or symptoms

Homozygous- problems are often severe or even fatal

40
Q

cause of thalassemias

A

when one of the Hb chains is not being made in adequate quantities, so a normal amount of Hb cannot be made –> excess globin chains accumulate within the erythrocyte and compromise cell structure and function –> abnormal erythrocytes recognized by spleen and selected for destruction (hemolysis)

41
Q

Thalassemia minor or major? one defective gene for the beta-globin molecule is inherited

A

minor

42
Q

Thalassemia minor or major? no significant clinical manifestations are usually present

A

minor

43
Q

Thalassemia minor or major? 2 defective genes for the beta-globin molecule

A

major

44
Q

another name for thalassemia major

A

Cooley’s or Mediterranean anemia

45
Q

develops in the first year of life because a severe microcytic, hypochromic anemia develops when fetal hemoglobin synthesis ceases after 3-4 months

A

thalassemia major

46
Q

Thalassemia minor or major? RBCs are extremely fragile and survive for only a few days

A

major

47
Q

Thalassemia minor or major? rate of hematopoiesis is greatly increased (up to 30x normal), although ineffective this is an attempt to maintain adequate oxygenation

A

major

48
Q

result of increased rate of hematopoiesis with thalassemia major

A
  • massive bone marrow hyperplasia

- hepatosplenomegaly from extramedullary hematopoiesis

49
Q

Thalassemia minor or major? reduced trabecular pattern of the mandible resulting from increased hematopoiesis

A

major

50
Q

Thalassemia minor or major?painless enlargement of the mandible and maxilla due to marrow hyperplasia

A

major

51
Q

tx of thalassemia major

A

blood transfusions administered every 2-3 weeks

52
Q

Result of repeated blood transfusions from tx of thalassemia major? What is particularly affected? Can lead to?

A
  • can lead to hemochromatosis- an abnormal deposition of iron throughout body tissues
  • heart, liver, and endocrine glands are particularly affected by the toxic accumulation of iron
  • can lead to death
53
Q

an abnormal deposition of iron throughout body tissues due to frequent blood transfusions

A

hemochromatosis (from tx of thalassemia major)

54
Q

failure of hematopoietic precursor cells in the marrow to produce enough of all types of blood cells

A

aplastic anemia

55
Q

How is aplastic anemia an immune-mediated disease?

A

cytotoxic T lymphocytes target hematopoietic cells in the marrow

56
Q

stem cells do not seem to undergo normal maturation despite normal or increased levels of cytokines

A

aplastic anemia

57
Q

Potential triggers of immune-mediated destruction (aplastic anemia):

A

Exposure to toxins

  • benzene exposures
  • chlororamphenicol (antibiotic no longer available orally in US)
  • certain viral infections (hepatitis nonA, nonB, nonC, nonG)
58
Q

3 things deficient with aplastic anemia and their side effects:

A

1) erythrocyte deficiency- decreased oxygen-carrying capacity of the blood, fatigue, lightheaded, tachycardia, weakness
2) thrombocytopenia- bruising and bleeding problems, retinal and cerebral hemorrhages
3) neutropenia, leukopenia, granulocytopenia- loss of WBCs, higher susceptibility to bacterial and fungal infections

59
Q

Oral manifestations:

  • pale mucosa due to decreased RBCs
  • gingival hemorrhage and hyperplasia
  • petechiae, purpura, and ecchymosis
A

aplastic anemia

60
Q

dx blood studies reveal pancytopenia (low granulocytes, platelets, and reticulocytes)

A

aplastic anemia

61
Q

tx of aplastic anemia

A
  • spontaneous recovery can occur in mild cases
  • antibiotics to prevent infections
  • transfusions of RBCs and platelets*
  • replace defective marrow* (bone marrow transplantation, peripheral blood stem cell transplantation)
62
Q

How do you replace defective marrow when treating aplastic anemia?

A
  • bone marrow transplantation

- peripheral blood stem cell transplantation

63
Q
  • increased susceptibility to bacterial infections

- caused by decreased production or increased destruction of neutrophils

A

neutropenia

64
Q

oral mucosal infections may be the first sign of this condition

A

neutropenia

65
Q

can be congenital/genetic or acquired

A

neutropenia

66
Q

acquired causes of neutropenia

A
  • malignancy crowds out and destroys normal marrow elements
  • medication induced
  • B12 deficiency
  • viral infections can increase destruction of neutrophils and decrease production of them (Hep A and B, rubella, measles, varicella, HIV)
  • some bacterial infections
67
Q

medications that cause neutropenia (4)

A
  • chemotherapeutics
  • antibiotics (penicillin, sulfonamides)
  • tranquilizers
  • diuretics
68
Q

Clinical findings:

  • pts tend to develop bacterial infections
  • gingival ulcers
  • premature periodontal bone loss with loss of primary teeth
A

neutropenia

69
Q

Bacterial infections that patients with neutropenia tend to develop and where?

A
  • Staph aureus and gram-neg organisms

- middle ear, oral cavity, and perirectal areas

70
Q

tx: antibiotic therapy, for oral involvement very good OH and frequent cleanings

A

neutropenia

71
Q
  • certain ethnic groups that consistently have neutrophil counts that would qualify as neutropenia (as low as 1200/mm3)
  • individuals are healthy
  • appears to have no effect on health of the patient
  • neutrophil counts respond to bacterial challenge
A

benign ethnic neutropenia (African and Middle Eastern)

72
Q

decreased # of circulating blood platelets

A

thrombocytopenia

73
Q

normal # of circulating blood platelets

A

200,000-400,000/mm3

74
Q

Causes of thrombocytopenia (3):

A

1) Reduced production- due to drugs or malignancy affecting marrow
2) Increased destruction- immunologic reaction to drugs, heparin, reaction to infection
3) Sequestration in the spleen- normally 1/3 of platelets are sequestered in the spleen, but some diseases lead to splenic enlargement and a greater number of platelets are retained there

75
Q

oral lesions are often initial finding, evident < 100,000 mm3, petechiae/ecchymosis/hematoma

A

thrombocytopenia

76
Q

If you see evidence of this in your patient, refer to their physician for evaluation.

A

thrombocytopenia

77
Q

Treatment:

  • If drug related: discontinue drug immediately, platelet count returns to normal after several days
  • Platelet transfusion
  • Possible corticosteroids
A

thrombocytopenia

78
Q

previous name for Langerhans cell histiocytosis

A

histiocytosis X

79
Q

proliferation of Langerhans cell histiocytes

A

Langerhans cell histiocytosis

80
Q
  • dendritic mononuclear cells found in the epidermis, mucosa, lymph nodes, and bone marrow
  • APCs, present antigens to T cells
A

Langerhans cell histiocytes

81
Q

clonality studies suggest this is a neoplastic disorder

A

Langerhans cell histiocytosis

82
Q

Why do studies suggest that Langerhans cell histiocytosis is a neoplastic disorder?

A
  • cells develop clonally –> grow from one “parent” cell

- with varying numbers of eosinophils, ly,phocytes, plasma cells and multinucleated giant cells

83
Q

> 50% of patients under age 15, 10-20% have jaw involvement

A

Langerhans cell histiocytosis

84
Q

affects 5 children and 1-2 adults per million population

A

Langerhans cell histiocytosis

85
Q

classifications of Langerhans cell histiocytosis

A

Unifocal disease (eosinophilic granuloma) –> single-system disease with single site of involvement

Multifocal disease (Hand-Schuller-Christian disease) –> multiple sites of involvement in single organ system

Multifocal multisystem disease (Leterer-Siwe disease) –> multiple involved sites in more than one organ system

86
Q

Name the classification of Langerhans cell histiocytosis: localized form, bony lesions without visceral involvement

A

unifocal disease (eosinophilic granuloma)

87
Q

Which form of Langerhans cell histiocytosis is this?

Clinical characteristics:

  • seen in older children and adults
  • asymptomatic or local pain
A

unifocal disease (eosinophilic granuloma)

88
Q

radiograph: RL areas, vary from punched out RL areas to ill-defined, teeth “floating in space”

A

Langerhans cell histiocytosis

89
Q

histo: mixed inflammatory cell infiltrate consisting of Langerhans histiocytes, variable numbers of eosinophils, lymphocytes, plasma cells, and neutrophils, has special stains to identify

A

Langerhans cell histiocytosis

90
Q

birbeck bodies/granules on SEM

A

Langerhans cell histiocytosis

91
Q

tx of Langerhans cell histiocytosis

A
  • depends on lesion size and degree of tissue involvement
  • surgery, radiation, chemotherapy
  • individually or in combination
  • surgery for accessible jaw lesions
  • possible intralesional injections of corticosteroids
92
Q

Neutropenia is a decrease in number of circulating neutrophils below _______.

A

1,500/mm^3