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Oral Pathology, Stephen > chpt 13- hematologic disorders > Flashcards

chpt 13- hematologic disorders Flashcards

1
Q

Proliferation of lymph cells in response to an antigenic challenge

A

Lymphoid hyperplasia

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2
Q

Areas of mouth for lymphoid hyperplasia

A

oropharynx
Soft palate
Lateral tongue
Floor of mouth

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3
Q

Acute vs Chronic lymphadenopathy

A

both are enlarged and freely movable, while the acute will also be soft while chronic is harder/rubbery

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4
Q

Variety of bleeding disorders associated with genetic deficiencies of any one of the clotting factors

A

Hemophilia

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5
Q

3 types hemophilia from most to least common

A

Hemophilia A
Hemophilia B
von Willebrand’s Disease

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6
Q

Hempophillia A is what factor deficiency

A

VIII

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7
Q

Hemophilia B is what factor deficiency

A

IX

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8
Q

von Willebrand’s disease is what deficiency

A

plasma glycoprotein

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9
Q

Hemophilia A is what type of genetic disorder and who is it expressed in

A

X-linked disorder with female carrier, but expressed in males

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10
Q

General term for a drop in the hematocrit or hemoglobin concentration

A

Anemia

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11
Q

general causes of anemia

A

blood loss

Decreased production of erythrocytes Increased destruction of erythrocytes

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12
Q

Anemia develops when…

A

not enough red blood cells(RBC) to transport oxygen to organs or RBC deficient in hemoglobin

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13
Q

What is the treatment of Hemophilia (I know this is out of place)

A

Replacement therapy with appropriate clotting factor

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14
Q

General symptoms of Anemia

A

tiredness, headache, lightheadedness (think of anything that will happen with low oxygen)

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15
Q

*****genetic disorder of hemoglobin synthesis that results from thymine substituting for adenine and eventually causing a rigid-curved(sickle) shaped cell instead of the normal biconcave shape

A

Sickle Cell Anemia

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16
Q

What tongue morphology is associated with vitamin deficiency and anemia

A

Bald tongue

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17
Q

Sickle Cell Anemia trait common in what demographic

A

Black and Mediterranean origin

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18
Q

Symptoms of sickle cell crisis

A

extreme pain due to infarction of tissue

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19
Q

Skull radiograph character of Sickle Cell

A

hair on end due to more marrow for extra hematopoiesis

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20
Q

2 disorders that cause Hair on End pattern

A
  • Sickle cell anemia

- Thalassemia

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21
Q

Sickle Cell anemia is prone to what bacterial infection

A

S. Pneumonia

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22
Q

Character of sickle cells

A

cannot pass through small blood vessels. Destroyed quicker than normal blood vessels

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23
Q

5 Things that can trigger sickling phenomenon

A 
exercise 
Exertion
Admin of general anesthetic 
Pregnancy
Sleep
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24
Q

Treatment of Sickle Cell Anemia

A

symptomatic and supportive therapy, oxygen, rest, fluids

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25
Q

Hemoglobin synthesis disorder characterized by reduced synthesis of either the alpha-globin or beta- globin chains of the hemoglobin molecule

A

Thalassemia

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26
Q

Homozygoud Thalasemia is major or minor and what does it cause

A

Major. Hemolytic anemia due to RBC membrane damage and destruction of RBC’s

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27
Q

Clinical look of Thalasemia major patient

A

*****prominent cheekbones, depressed nasal bridge, prominent premaxilla, flaring of maxillary anterior teeth

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28
Q

Key trait on radiograph to associate with Thalassemia

A

Thinning of Lamina dura

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29
Q

Thalassemia treatment

A
  • blood transfusions and deferoxamine infusions nightly.

- Splenectomy

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30
Q

**Rare, life-threatening anemia caused by the failure of hematopoietic prescursor cells in bone marrow to produce adequate numbers of all types of blood cells

A

Aplastic Anemia

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31
Q

Aplastic anemia cause of death

A

White Blood Cell (WBC) deficiency (neutropenia, leucopenia, granulocytopenia) leads to bacterial and fungal infections

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32
Q

Granulocyte, platelet, reticulocyte numbers to diagnose Aplastic Anemia

A

granulocytes <20,000

Normal Granulocytes/neutrophiles = 6,000-10,000

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33
Q

Bone marrow character of aplastic anemia

A

relatively acellular with extensive fatty infiltration

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34
Q

Best treatment for Aplastic Anemia

A

patients younger than 40 with HLA matched bone marrow donor

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35
Q

***Decrease in the number of circulating neutrophils below 1500/mm3

A

Neutropenia

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36
Q

2 mechanisms that cause neutropenia

A

decreased production

Increased destruction

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37
Q

What nutritional deficiency can cause neutropenia

A

*****Vit B12 or folate deficiency

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38
Q

Bacteria susceptible to with neutropenia

A

S. aureus and gram negatives

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39
Q

Oral character of neutorpenia

A

gingival ulcers lacking characteristic erythmatous halo

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40
Q

**Neutropenia treatment

A

Cytokine granulocyte colony stimulating factor

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41
Q

**Condition where the cells of the granulocytic series, particularly neutrophils, are absent, through either decreased production or increased destruction

A

Agranulocytosis

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42
Q

Oral lesions common with Agranulocytosis

A

**Ulcerations and Necrotizing Ulcerative Ginigivitis (NUG)

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43
Q

**Agranulocytosis treatment

A

if suspect drug-induced, stop drugs. Give Granulocyte-macrophage colony stimulating factor (GM-CSF)

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44
Q

**Rare idiopathic hematological disorder that is characterized by regular periodic reductions in neutrophil population

A

cyclic neutropenia

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45
Q

Normal cycle for cyclic neutropenia

A

approximately 21 day cycle

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46
Q

Symptoms of cyclic neutropenia

A

seem to have flu every 3 weeks dor 3-6 days

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47
Q

character of ulcers in cyclic neutropenia

A

**have erythmatous halo

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48
Q

**Decreased number of circulating blood platelets due to either decreased production, increased destruction or sequestration in the spleen

A

Thrombocytopenia

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49
Q

Normal platelet count

A

200,000 – 400,000/ mm3

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50
Q

Platelets required for what

A

hemostasis and clot formation

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51
Q

Things that can cause reduced platelet production

A

Malignancy of bone marrow

Toxic effects of cancer chemotherapy

52
Q

2 systemic diseases that can cause thrombocytopenia

A

Systemic lupus (SLE) and HIV

53
Q

At what platelet count is thrombocytopenia seen

A

<100,000/mm3

54
Q

Thrombocytopenia can cause what lesions

A

**petechia, ecchymosis, hematoma

55
Q

Clinical signs of thrombocytopenia

A

-epistaxis (nose bleeds)
-Hemoptosis (vomiting blood = pulmonary
hemorrhage)

56
Q

**Thrombocytopenia occurring in childhood after a nonspecific viral infection with quick and sever onset but can self resolve in 4-6 weeks

A

Idiopathic (immune) Thrombocytopenic purpura (ITP)

57
Q

**Serious disorder of coagulation thought to be caused by endothelial damage triggering formation of numerous thrombi within small blood vessels of body

A

Thrombotic Thrombocytopenic purpura (TTP)

58
Q

Thrombocytopenia treatment

A

discontinue offending drug and platelets return in a few days

59
Q

Idiopathic thrombocytopenic purpura treatment

A

spontaneous resolution or give corticosteroids or IV immunoglobulin

60
Q

Thrombotic thrombocytopenic purpura (TTP) treatment

A

plasma pheresis or plasma exchange. Still have guarded prognosis, but no longer certain death

61
Q

**Idiopathic hemorrhagic disorder caused by increase in mass of Red Blood Cells

A

Polycythemia Vera

62
Q

Peripheral vascular event affect hands and feet of pt with Polycythemia Vera where there is a painful burning sensation accompanied by erythema and warmth that may eventually lead to thrombotic occlusion of vessels supplying digits

A

Erythromelalgia

63
Q

Gen mutation in 95% of Polycythemia Vera

A

Tyrosine kinase gene mutation

64
Q

40% of Polycythemia Vera patients complain of this after a bath

A

**itching with absence of rash

65
Q

**3 treatments of Polycythemia Vera

A

1) Phlebotomy (500 ml blood drain/day)
2) Anagrelide HCl to control platelet levels
3) Antihistamines for purities (itching)

66
Q

Term for several types of malignancies of hematopoietic stem cell derivation

67
Q

Syndromes with increased risk of Leukemia

A
  • Downs
  • Neurofibromatosis 1
  • Klinefelter’s (XXY)
  • Fanconi’s Anemia
68
Q

Which leukemia affects children and adults

A

Acute Myeloid leukemia

69
Q

Which leukemia peaks in the third and fourth decade

A

Chronic myeloid

70
Q

Which leukemia is almost always in children

A

Acute lymphoblastic leukemia

71
Q

Which leukemia primarily affects elderly adults

A

Chronic Lymphocytic Leukemia

72
Q

malignant proliferation in bone marrow with leukemia causes what anemia

A

myelophthisic anemia

73
Q

As marrow is crowded out in leukemia what happens to RBC

A

reduced oxygen capacity = fatigue

74
Q

When platelets drop below 20,000 mm3 (thrombocytopenia induced by leukemia ) what is seen on the posterior hard and soft palates

75
Q

Leukemia gingival

A

**boggy gums

76
Q

6 treatments for Leukemia

A

1) drugs + radiation
2) Bone marrow transplant nt <45
3) Platelet transfusion
4) Packed Red blood cells for anemia
5) Antibiotics for infection
6) Optimal oral hygiene

77
Q

4 types of Leukemias and their age ranges

A
  • Acute Myeloid Leukemia (adults & children)
  • Chronic Myeloid Leukemia (30-40 year olds)
  • Acute Lymphoblastic Leukemia (children)
  • Chronic Lymphocytic Leukemia (older adults)
78
Q

Most common Leukemia and is it considered curable

A

Chronic Lymphocytic Leukemia. Not curable

79
Q

Cause of death in Chronic Myeloid Leukemia

A

Blast transformation after long indolent period

80
Q

Recognize histology slide with large white cells indicating leukemia beside a picture of someone with inflamed gingiva

81
Q

Dendritic mononuclear cells found in the epidermis, mucosa, lymph nodes, and bone marrow acting as antigen presenting cells to T-cells

A

Langerhans cells

82
Q

*****Spectrum of disorders characterized by a proliferation of histiocyte-like (Langerhans cells) cells that are accompanied by varying numbers of eosinophils, lymphocytes, plasma cells, and multinucleated giant cells. Shows monoclonal proliferation as would be seen in neoplastic process

A

Langerhans cell histiocytosis

83
Q

3 types of Langerhans Cell Histiocytosis

A

-Monostotic or polyostotic Eosinophilic Granuloma
-Hand Schuller Christian Disease/ Chronic
Disseminiated histiocytosis
-Letterer-Siwe Disease/Acute Disseminated
histiocytosis

84
Q

What is the most common Langerhans Cell histiocytosis

A

Hand Schuller Christian Disease/ Chronic Disseminiated histiocytosis

85
Q

**Most common clinical presentation of Langerhans Cell Histiocytosis

A

Bone lesions

86
Q

Radiographic appearance of Langerhans Cell histiocytosis in jaw

A

*****Tooth floating in air appearance

87
Q

**Malignant lymphoproliferative disorder usually beginning with lymph nodes presenting as persistently enlarging, nontender mass or masses in one lymph node region

A

Hodgkin’s Lymphoma

88
Q

Characteristic cells of Hodgkin’s lymphoma

A

**Reed-Sternberg cells (owl eyes)

89
Q

Hodgkin’s lymphoma found 75 % in what lymphnodes

A

Cervical and supraclavicular nodes

90
Q

Hodgkin’s lymphoma treatment

A

based on staging. Stage I & II use mild radiation and multiagent chemo. Stage III & IV use chemo

91
Q

Most common histologic type of Hodgkin’s lymphoma

A

Nodular Sclerosis (frequent in 20 year old females)

92
Q

**Group of malignancies of lymphoreticular histogenesis arising in the lymph nodes and growing in a solid mass

A

Non-Hodgkin’s lymphoma

93
Q

Non-Hodgkin’s lymphoma 85% arise from what lymphocyte

94
Q

3 categories of Non-Hodgkin’s lymphoma

A

Low grade
Median grade
High grade

95
Q

Non-Hodgkin’s Lymphoma in bone character

A

will be ill defined/ ragged radiolucency w/ expansion & cortical perforation

96
Q

Non-Hodgkin’s lymphoma oral common sites

A

Buccal vestibule, posterior hard palate, gingival

97
Q

What can Non-Hodgkin’s lymphoma in jaw bone be mistaken for

A

**toothache

98
Q

Numb chin syndrome in Non-Hodgkin’s lymphoma why

A

mandibular lesion hitting IAN

99
Q

Non-Hodgkin’s lymphoma treatment

A

all with radiation &/or chemo. Low grade recurs, Intermediate and High grade responds, but low cure rate

100
Q

CD 4 T cell lymphoma, but sounds like a fungal infection

A

Mycosis Fungoides

101
Q

Demographics for Mycosis Fungoides

A

2:1 male, 55-60

102
Q

3 stages of Mycosis Fungoides

A

Eczematous, plaque, tumor

103
Q

Which stage of Mycosis Fungoides can be confused with psoriasis

A

eczematous

104
Q

Aggressive expression of T Cell Leukemia

A

Sezary syndrome

105
Q

Cure rate of Mycosis Fungoides

A

not curable, but usually slowly progressive

106
Q

**Malignancy of B-lymphocyte origin that represents an undifferentiated lymphoma

A

Burkitt’s Lymphoma

107
Q

Burkitt’s Lymphoma is related pathogenetically to what virus

A

**Epstein Barr Virus

108
Q

Demographics and location of Burkitt’s Lymphoma

A

Children ~ 7 yrs, 50-70% in posterior jaws, Maxilla>Mandible

109
Q

Jaw involvement of Burkitt’s Lymphoma is based on what

A

age. 3 yrs old, 90% have jaw lesion vs. >15 years old, 25% have jaw lesion

110
Q

Radiographic character of Burkitt’s lymphoma

A

**ragged borders, patchy loss of lamina dura

111
Q

Histo pattern of Burkitt’s Lymphoma

A

**Starry sky

112
Q

Burkitt’s lymphoma treatment

A

intense chemo with cyclophosphamide w/ 90% disease free. Death w/in 4-6 months if untreated

113
Q

**Malignancy of plasma cell origin that often appears to have a multicentric origin within bone

A

Multiple Myeloma

114
Q

Demographics and symptoms of Multiple Myeloma

A

40 year old black males w/ bone pain

115
Q

Radiographic appearance of Multiple myeloma and jaw incidence

A

**“punched out” boney lesions. 30% jaw

116
Q

other common presenting symptom beyond bone pain of Multiple Myeloma and the urine protein used to diagnose

A

Renal failure due to kidney overburden w/ light chains. Bence Jones Proteins = circulating light chains

117
Q

Multiple Myeloma tongue character due to amyloid

A

enlarged, firm, nodular

118
Q

Histo of Multiple Myeloma

A

malignant plasma cells w/ eccentric nuclei and stippled nuclear chromatin in sheets indicating monoclonal origin

119
Q

Treatment Multiple Myeloma

A

Aggressive chemo and bone marrow transplant if able. 25% 5 year survival if chemo only. 50% survival if chemo and bone marrow transplant

120
Q

**Unifocal, monoclonal, neoplastic proliferation of plasma cells in bone (versus the multcentric origin of Multiple Myeloma)

A

Plasmacytoma

121
Q

Demographics for Extramedullary Plasmactyoma

A

6:1 Males, 90% head and neck

122
Q

Most common site for Plasmacytoma

123
Q

What may Plasmacytoma progess to

A

Multiple Myeloma

124
Q

Treatment of Plasmacytoma

A

radiation at least 4000 cGy

125
Q

Which has better prognosis Plasmacytoma or Extramedullary Plasmacytoma

A

Extramedullary plasmacytosis, 70% 10 year disease free w/ 30% convert to multiple myeloma

126
Q

Radiographically, is the plasmacytoma a single or multiple punched out bony lesions

A

**Single punched out boney lesion

Oral Pathology, Stephen (13 decks)

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