Lecture Flashcards
1
Q
Four oral cavity sites for normal accessory lymphoid tissues
A
- Floor of mouth
- Soft Palate
- Posterior lateral border of tongue
- Ventral tongue
2
Q
Can lymphoid hyperplasia be acute or chronic?
A
Both
3
Q
What areas can lymphoid hyperplasia affect?
A
Lymph nodes
Lymphoid tissue of Waldeyer ring
Aggregates of lymphoid tissue that are normally scattered throughout the oral cavity
4
Q
Lymphoid Hyperplasia Intraoral Clinical Appearance
A
Discrete, non-tender, submucosal swelling, <1cm, multifocal
5
Q
Should we biopsy lymphoid hyperplasia?
A
Does not require biopsy unless:
- Progressive enlarging -> biopsy to rule out lymphoma
- Overgrowth effects patient function
6
Q
What should be done with progressive asymmetric enlargement of tonsillar tissue or cervical lymph nodes?
A
-Consider referral to ENT for evaluation/work-up to rule out lymphoma or other malignancy
7
Q
Hemophilia A Inheritance Pattern
A
X-linked recessive
8
Q
Hemophilia A Etiology
A
Mutation in gene responsible for clotting Factor 8
9
Q
Hemophilia A:
The greater proportion of gene mutation=
A
Worse disease
10
Q
Hemophilia A:
Patients with _____% or greater Factor 8 production live fairly normal lives
A
25%
11
Q
Hemophilia A:
Patients with ___% bruise with minor trauma
A
5%
12
Q
Hemophilia A: Lab Values
A
- Abnormal PTT
- Usually normal PT and platelets
13
Q
Hemophilia B:
Inheritance Pattern
A
X-linked recessive
14
Q
Hemophilia B:
Alternative name
A
Christmas Disease
15
Q
Hemophilia B:
Etiology
A
Factor 9 Deficiency
16
Q
Hemophilia B:
Lab Values
A
Abnormal PTT
-Usually normal PT and platelets
17
Q
What is the most common bleeding disorder?
A
Von Willebrand Disease
18
Q
Von Willebrand Disease:
Inheritance Pattern
A
Usually autosomal dominant
19
Q
Von Willebrand Disease:
Etiology
A
Abnromal von Willebrand factor and abnormal platelets
20
Q
Define Von Willebrand Factor
A
Glycoprotein in plasma that aids in adhesion of platelets to a bleed site
Factor also binds to factor 8 and acts as a transport molecule
21
Q
Von Willebrand Disease:
Lab values
A
Abnormal PTT and platelet function
22
Q
What are two major oral path considerations with hemophilia and VWD?
A
- Pseudo tumor of hemophilia
- Bleeding risk if biopsy is needed
23
Q
Describe a pseudotumor of hemophilia
A
Hemorrhage within the bone or soft tissue that produces a tumor like clinical swelling
24
Q
What may be needed in the med consult before a biopsy in a hemophilia or VWD patient?
A
- Assess risk for bleeding
- Replacement of clotting factor
- Epsilon-aminocaproic acid
- Desmopressin
25
If a biopsy is needed in hemophilia or VWD patient, what type of closure is needed?
Primary Closure
26
Plasminogen Deficiency:
| Inheritance Pattern
Rare; autosomal recessive inheritance
27
Plasminogen Deficiency:
| Mutation
Mutation in gene for plasminogen
28
Plasminogen Deficiency: Why is plasminogen important?
Necessary for breaking down cloths as part of normal healing.
Without this -> clot grows and persists
29
Plasminogen Deficiency:
| Clinical Presentation
Irregular fibrin deposited in plaques and nodules primarily in the mucosal surface
30
Plasminogen Deficiency:
| Treatment of oral lesions
- Med consult & partnership
- Topical plasminogen not commercially available
- Topical heparin and prednisone combined
- Surgical excision of large nodules + systemic doxyclycline, systemic warfarin, topical chlorhexidine
31
Plasminogen Deficiency:
| Life expectancy; what does the patient not suffer from?
Normal; patient does not have intravascular thrombus formation
32
Aplastic Anemia:
| Rare, _____-threatening hematologic disorder
Life
33
Aplastic Anemia:
| Characterized by:
Failure of hematopoietic precursor cells in the bone marrow to produce adequate numbers of all types of blood cells
34
Aplastic Anemia:
| Describe Pancytopenia
less than 500 granulocytes, 20,000 platelets and 20,000 reticulocytes
35
Aplastic Anemia:
| Etiology
Immune-mediated destruction
36
Aplastic Anemia:
| Triggers
Not fully understood
37
Aplastic Anemia:
| Two associated inherited conditions
- Fanconi anemia
| - Dyskeratosis congenita
38
Aplastic Anemia:
| Clinical characteristics
-Thrombocytopenia: Gingival hemorrhages - petechiae, purpura ecchymosis
Neutropenia: gingival ulcers
39
Aplastic Anemia:
| Treatment/Prognosis
-Systemic therapy: initially palliative, eventually bone marrow transplant/peripheral blood stem cell transplant
40
What occurs in agranulocytosis?
Cells of the granylocyte lineage are absent
| Particularly neutrophils
41
Agranulocytosis:
| Etiology; when do symptoms appear?
Caused by a drug. Symptoms begin a few days after drug ingestion.
42
Agranulocytosis:
| Clinical Features
- Related to increase in infection risk
- Fever, chills, malaise
- Oral Lesions: Deep, nectrotizing ulcerations, gingiva prone to infection that resembles NUG
43
Agranulocytosis:
| Treatment
- Discontinuation of offending drug unless drug is a necessity
- Oral hygeine, antibiotic therapy as necessary, consider chlorhexidine mouth wash
44
Cyclic Neutropenia:
| What occurs?
Idiopathic reductions in the neutrophil count of affected patients that occur as uniformly spaced episodes
45
Cyclic Neutropenia is caused by what type of mutation?
A specific genetic mutation in neutrophil elastase gene
46
When is cyclic neutropenia noticed?
In childhood
47
General symtpoms of cyclic neutropenia
Fever, anorexia, cervical LAD, malaise, pharyngitis, lower GI ulcers
48
Oral Symptoms of Cyclic neutropenia
- Oral ulcers that occur anywhere with minor trauma
| - Severe periodontal bone loss and gingival recession
49
Cyclic Neutropenia Diagnosis
Sequential blood counts; neutrophil count falls to 500 for 3-5 days in at least 3 sucessive cycles for diagnosis
50
Cyclic Neutropenia Treatment
- Optimal oral hygeine to reduce risk for infection
- Can consider topical steroids for painful oral ulcers
- Systemic therapies are used by medical colleagues
- Severity of symptoms usually diminishes after the second decade of life (even though the cycling is still occurring)
51
Define Amyloidosis
Group of conditions characterized by deposition of an abnormal protein called amyloid
52
Where may amyloid deposit? What does it cause?
In multiple vital organs (heart, brain, kidneys, liver); significant dysfunction
53
What are two types of Amyloidosis?
Organ-limited/localized Amyloidosis
| Systemic Amyloidosis
54
Amyloidosis:
| Describe Organ-limited/localized Amyloidosis
- Single focus of disease
| - Limited or no complications
55
Amyloidosis:
| Describe systemic Amyloidosis
-Multifocal/widespread disease
56
Primary & Myeloma Associated Amyloidosis:
| Etiology
Primary: Idiopathic
| Myeloma Associated: Caused by patient having the condition multiple myeloma
57
Primary & Myeloma Associated Amyloidosis:
| What is the abnormal light chain associated with these conditions?
AL Amyloidosis
58
Primary & Myeloma Associated Amyloidosis:
| Population affected
Older adult -> 65
| Slight male predilection
59
Primary & Myeloma Associated Amyloidosis:
| Initial Symptoms
Nonspecific; fatigue, weight loss, hoarseness
60
Primary & Myeloma Associated Amyloidosis:
| Later signs
Carpal tunnel, mucocutaneous lesions, hepatomegaly, macroglossia, xerostomia and xerophthalmia
61
Head and Neck Involvement by Amyloid:
| Skin Characteristics
- Smooth-surfaced, firm, waxy papules and plaques +/- petechiae and or ecchymosis
- Most commonly affect the eyelids, neck, lips
62
Head and Neck Involvement by Amyloid:
| Describe amyloid lesions
- Similar to the skin
- Smooth surfaced, firm, waxy papules and plaques +/- petechiae and or ecchymosis
- Nodules/plaques may ulcerate and show submucosal hemorrhage
63
Head and Neck Involvement by Amyloid:
| Describe population and presentation of macroglossia
Reported in 10 to 40% of patients
| Diffuse or nodular enlargement of the tongue
64
Describe Secondary Amyloidosis
Chronic inflammatory disease results in over production of serum amyloid A
65
What amyloid protein creates hemodialysis associated amyloidosis
B2-microglobulin
66
What occurs in hemodialysis associated amyloidosis?
Microglobulin normally occurs in the body and is not removed by dialysis -> accumulation and formation of deposits particularly in bone and joints
67
Heredofamilial amyloidosis is an _____ disorder
Inherited
68
Systemic Amyloidosis:
| If caused by underlying inflammatory disease (secondary amyloidosis) treat ->
Systemic disease
69
Systemic Amyloidosis:
| If caused by need for hemodialysis ->
Kidney transplant
70
Systemic Amyloidosis:
| If idiopathic or myeloma associated ->
Systemic medications have shown efficacy
71
Systemic Amyloidosis:
| Long term prognosis
Poor
| -Most patients die of cardiac failure, arrhythmia or renal disease within months to a few years after diagnosis
72
Describe Thrombocytopenia
Marked decrease in circulating platelets
73
Thrombocytopenia:
| Three main etiologies
- Decreased production
- Increased destruction
- Increased use of platelets
74
Thrombocytopenia:
| Described decreased production
Decreased production in the bone marrow from malignant infiltration or chemotoxicity
75
Thrombocytopenia:
| Describe increased destruction
Introduction of an offending agent (ex. viral infection) -> immunologic reaction -> autoantibodies to platelet surface glycoproteins
76
Thrombocytopenia:
| What two diseases can cause increased destruction of platelets?
SLE and HIV
77
Thrombocytopenia:
| Describe why there is an increased use of platelets
- Disorders of abnormal blood clot formation
| - Disorders than cause splenomegaly (increased platelet trapping in the spleen)
78
Thrombocytopenia: When are symptoms usually noticeable?
When platelets are below 100,000
79
Thrombocytopenia: What is the first sign?
Oral lesions
80
Thrombocytopenia: How are oral lesions most commonly created?
Minor trauma from eating causes petechiae and they can have spontaneous gingival hemorrhage from minor trauma sites
81
Thrombocytopenia:
| Mucosa and skin may show what three signs?
Petechiae, ecchymosis and hematoma
82
Thrombocytopenia:
| Other signs and symptoms
Epistaxis, hemoptysis, GI bleeding, urinary bleeding, intracranial hemorrhage
83
Thrombotic Thombocytopenic Purpura TTP:
| Define
Increased use of platelets which results in thrombocytopenia
84
Thrombotic Thombocytopenic Purpura TTP
| Caused by:
Deficiency in von Willebrand factor cleaning metalloprotease (ADAMTS13)
85
Thrombotic Thombocytopenic Purpura TTP Triggers formation of what?
Numerous thrombi within small vessels
86
Thrombotic Thombocytopenic Purpura TTP:
| Inheritance pattern/cause
Either from autoantibodies to ADAm or can be inherited -> autosomal recessive when ADAm is mutated
87
Thrombotic Thombocytopenic Purpura TTP
| Treatment
With adequate systemic therapy -> 70 percent survial rate
88
Immune Thrombocytopenic Purpura (ITP):
| Define
Increased destruction of platelets results in thrombocytopenia.
89
Immune Thrombocytopenic Purpura (ITP):
| Etiology
Autoantibodies directed against antigens on the platelet surface result in sequestration and destruction of the platelets in the spleen
90
Immune Thrombocytopenic Purpura (ITP):
| Acute or chronic?
Both
91
Immune Thrombocytopenic Purpura (ITP):
| Describe population affected by acute ITP
Children following non-specific infection
92
Immune Thrombocytopenic Purpura (ITP): Describe how long acute ITP occurs
4-6 weeks
| 90% of patients recover in 3-6 months
93
Immune Thrombocytopenic Purpura (ITP):
| Describe population affected by chronic ITP
Older patients; women; 20-40 years old
94
Immune Thrombocytopenic Purpura (ITP): Treatment
Frequently treated with splenectomy
95
Define Lymphoproliferative disorder
Disease in which cells of the lymphatic system grow excessively
96
Lymphoproliferative disorder: Name the types of cancers associated
Leukemia, lymphoma, multiple myeloma
97
Monoclonal cell population arises from what?
A single progenitor cell
98
Polyclonal cell population arises from what?
Two or more distinct progenitor cells
99
Describe what occurs in B symptoms
Unexplained fever, night sweats, greater than 10 percent involuntary weight loss
100
What are langerhans cells?
Dendritic mononuclear cells that process and present antigens to T cells
101
Langerhans cell histiocytosis (LCH):
| Describe
Monoclonal proliferation of langerhan cells
102
Langerhans cell histiocytosis (LCH):
| Cause
Gene mutation (BRAF in 40 to 60%)
103
Langerhans cell histiocytosis (LCH):
| Two types of classifications
- Single organ involvement
| - Multi organ involvement
104
Langerhans cell histiocytosis (LCH):
| Where does single organ involvement usually occur?
Bone or skin; can be unifocal or multifocal
105
Langerhans cell histiocytosis (LCH):
| Describe two types of multi-organ involvement
No organ dysfunction
| Organ dysfunction
106
Langerhans cell histiocytosis (LCH):
| Describe what areas are affected in low risk organ involvement
Skin, bone, lymph nodes, pituitary gland
107
Langerhans cell histiocytosis (LCH): Describe what areas are affected in high risk organ involvement
Lung, liver, spleen, bone marrow
108
Langerhans cell histiocytosis (LCH):
| Describe population affected
More than 50% of cases are patients younger than 15
109
Langerhans cell histiocytosis (LCH):
| Hard Tissue manifestations
Jaw is affected in 10-20 percent of cases
110
Langerhans cell histiocytosis (LCH): Jaw presentation in radiographs
Radiolucent, sharp, punched-out or "scooped out" bony lesions
-Posterior mandible most often affected
111
Langerhans cell histiocytosis (LCH): Can mimic what disease?
Periodontal disease
112
Langerhans cell histiocytosis (LCH):
| Describe soft tissue manifestations
Gingival mass lesion with ulceration
113
Langerhans cell histiocytosis (LCH):
| Treatment with localized disease
- Accessible lesions are curretted (ensure there are no other organs involved)
- Low dose radiation used for non-acessible bone lesions
- Corticosteroid infections
- Prognosis of local disease is good
114
Langerhans cell histiocytosis (LCH):
| Treatment with multiorgan involvement
- Low risk pattern -> significant morbidity, but much lower mortality than high risk patterns
- Treated with systemic chemotherapy and prednisone combination
- Younger onset of disease -> worse prognosis
115
Rosai-Dorfman Disease:
| Alternative Name
Sinus Histiocytosis with Massive Lymphadenopathy
116
Rosai-Dorfman Disease:
| Describe
Rare proliferative disorder of histiocytes
117
Rosai-Dorfman Disease:
| Proliferation of histiocytes may involve?
- Lymph nodes
| - Extranodal sites: skin, oral cavity, nasal cavity, respiratory tract, eyelids, salivary glands, bone
118
Rosai-Dorfman Disease:
| Describe population affected
Children and young adults; males
119
Rosai-Dorfman Disease: Presenting symptoms
-Massive, non-tender bilateral cervical lymph node enlargement, fever
120
Rosai-Dorfman Disease: Oral manifestations
Ill-defined radiolucency of bone; bony destruction
- Bone is infiltrated with abnormal histiocytes
- May mimic periodontal disease or periapical pathology
- Associated bone resorption may cause tooth mobility
121
Rosai-Dorfman Disease: Treatment/Prognosis
- Non-standardized: Excision of oral lesions without recurrence.
- Systemic treatments: variable, include corticosteroids, chemotherapy agents, radiation
122
Polycythemia Vera:
| Define
Rare idiopathic myeloproliferative disorder
123
Polycythemia Vera: Results in over production of what?
Blood cells in the bone marrow
124
Polycythemia Vera: What population does this occur in?
Older adults; 60 years old
125
Polycythemia Vera: Etiology
Acquired mutation in JAK2
126
Polycythemia Vera: General symptoms
Headaches, weakness, dizziness, sweat, weight loss, epigastric pain
127
Polycythemia Vera:
| 40% of patients have what symptom?
Generalized pruiritis w/o rash
128
Polycythemia Vera: An increased viscosity of blood from excess blood cells puts patients at increased risk for what two conditions?
Cerebrovascular accident and MI
129
Polycythemia Vera: Symptom causing painful burning sensation, erythema and warmth of hands and feet
Erythomelalgia
130
Polycythemia Vera: Can cause loss of circulation causing ______
Gangrene
131
Polycythemia Vera: Oral Symptom
Gingival hemorrhage
132
Polycythemia Vera: Treatment
Phlebotomy, low dose aspirin, myelosupressive therapy
133
Leukemia: Signs and symptoms
Myelophthisic anemia
134
Leukemia: Diagnostic techniques
Peripheral blood smear, flow cytometry, bone marrow biopsy
135
Leukemia: Intraoral symptoms
- ulceration and infection more common
- Leukemic cells can infiltrate the gingiva
- Myeloid sarcoma
136
Define myeloid sarcoma
Tumor-like growth of leukemia cells
137
Define lymphoma
-Malignant transformation of cells of the B and T cell lineages.
138
Where does lymphoma arise?
-Initially arise within lymph nodes and tend to grow as solid masses.
139
Hodgkin Lymphoma:
| Signs and symptoms
- Persistent enlarging, nontender, massess in a lymph node region
- Cervical and supraclavicular nodes
140
Hodgkin Lymphoma: Diagnostic techniques
Lymph node biopsy
141
Non-hodgkin Lymphoma:
| Describe beginning stages
Proliferation in the lymph nodes but can expand to other body sites
142
Non-hodgkin Lymphoma: What is the most frequent oral site being affected?
Palate
143
Non-hodgkin Lymphoma: Describe oral lesions in the palate
- Intraoral lesions most commonly present as red/purple/blueish "boggy" swellings
- Telengiectasias overlying the affected site is note uncommon
144
Non-hodgkin Lymphoma: Four subtypes
- DLBCL
- MALT definition
- Burkitt lymphoma
- Mycosis fungoides skin
145
Diffuse Large B Cell Lymphoma: Describe
- High grade Lymphoma
| - Often represents transformation of another low-grade lymphoma
146
What is the most common lymphoma affecting the oral cavity?
Diffuse large B cell lymphoma
147
MALT Lymphoma: Describe
B Cell NHL that specifically occurs outside of the lymph nodes
148
MALT Lymphoma affects what areas most commonly?
Stomach and in patients with chronic inflammation (Such as H. Pylori infection)
149
MALT Lymphoma Can also occur in what places?
Small intestine, salivary glands, thyroid, eyes, lungs
150
Patients with what syndrome have a 20x increased risk for developing lymphoma?
Sjogren Syndrome
151
Mycosis Fungoides:
| Describe
Cutaneous T cell lymphoma; most common cutaneous lymphoma
152
Mycosis Fungoides:
| Oral Cavity Complications
Rarely can affect the oral cavity
| Erythametous, indurated plaques or nodules that frequently are ulcerated
153
What is Sezary syndrome?
Aggressive form of mycosis fungoides
154
Multiple Myeloma: Etiology
Hematologic malignancy of plasma cells
155
Describe Plasmacytoma
If there are only 1 tumor-like mass of malignant plasma cells
156
Multiple plasmacytomas=
Multiple myeloma
157
Multiple Myeloma Diagnosis
- Serum eletrophoresis
- Biopsy of bony or soft tissue lesions
- Urine test
158
Multiple Myeloma Oral Findings
-Multiple punched out radiolucencies of the jaw bones
