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GENERAL OTOLARYNGOLOGY > CHAPTER 10: Head and Neck Manifestations in the Immunocompromised Host > Flashcards

CHAPTER 10: Head and Neck Manifestations in the Immunocompromised Host Flashcards

1
Q

Protection of the host occurs by two mechanisms

A
  1. Innate

2. Adaptive

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2
Q

First line of defense

A

Innate immune system

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3
Q

Primary cells involved in innate immune system

A
  1. Neutrophils
  2. Eosinophils
  3. Basophils
  4. Macrophages/monocytes
  5. Dendritic cells
  6. NK cells
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4
Q

Responsible for protecting the host against pathogens that escape innate immunes responses

A

Adaptive immune system

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5
Q

Cellular components of adaptive immunity

A

T and B lymphocytes

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6
Q

Less common than secondary immunodeficiencies, rarely affect the innate immune system

A

Primary immunodeficiencies

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7
Q

Affects both the T and B cells of the adaptive immune system

A

Severe combine immunodeficiency

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8
Q

Characteristics of T-cell dysfunction include

A

Onset of symptoms in early infancy with recurrent fungal, viral, mycobacterial, and opportunistic infections (Pneumocystis jirovecii)

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9
Q

Marked by pyogenic bacterial infections with encapsulated organisms

A

B-cell antibody deficiencies

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10
Q

Key role in DM

A

Neutrophil dysfunction—>functional neutrophil deficiency

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11
Q

True or False
Patients with DM demonstrate impaired neutrophil chemotaxis and phagocytic function that improves with insulin treatment and reversal of hyperglycemia

A

True

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12
Q

Defect: Lack of bacterial opsonization

Hallmark infection/signs: encapsulated organisms

A

C3

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13
Q

Defect: terminal complement deficiency

Hallmark infection/signs: Neisseria meningitidis

A

C5-C9

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14
Q

Defect: decrease in at least 2 Ig and defective antibody production
Hallmark infection/signs: encapsulated organisms, poor vaccination responses, increased risk of bronchiectasis at diagnosis

A

Common variable immunodeficiency

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15
Q

Defect: thymic hypoplasia/T-cell deficiency

Hallmark infection/signs: variable

A

22q11.2 deletion (DiGeorge/Velocardiofacial) syndrome

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16
Q 
Defect: one or more deficient Ig class
Hallmark infections/signs: variable
A

Selective Ig deficiencies

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17
Q

Defect: severe B and T cell deficiency, lymphopenia

Hallmark infection/signs: severe, early-onset bacteria, viral, and fungal infections

A

Severe combined immunodeficiency

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18
Q

Defect: WASP gene defect—>neutropenia, T cell lymphopenia

Hallmark infection/signs: thrombocytopenia, recurrent infection, and eczema

A

Wiskott-Aldrich syndrome

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19
Q

Defect: BTK gene defect—>Failure of B-lymphocyte maturation and decreased Ig and specific antibody production
Hallmark infection/signs: encapsulated organism, Giardia lambia, and enterovirus infections

A

X-linked agammaglobulinemia

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20
Q

Is diagnosed when CD4 count drops below 200 cells/mm or patients develop OI not normally seen in immunocompetent patients

A

AIDS

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21
Q

Is a retrovirus of the Lentivirus subfamily, named for the slow progression of disease in affected individuals

A

HIV

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22
Q

Affected cells in HIV resulting in both humoral and cell-mediated immunity

A

CD4 T cells and macrophages

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23
Q

Virus life cycle begins when…

A

Binds to the CD4 receptor

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24
Q

An enzyme carried by the virus, allows transcription of RNA into DNA

A

Reverse transcriptase

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25
Q

A viral protein that facilitates incorporation of the viral DNA into the host genome

A

Integrase

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26
Q

Required for viral infectivity

A

Protease

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27
Q

Major goals of ART

A
  1. Prevention of virus entry into CD4 cells
  2. Inhibition of viral replication
  3. Reduction in HIV-associated morbidity
  4. Prevention of vertical transmission
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28
Q

Five classes of ART

A
  1. Nucleoside/nucleotide reverse transcriptase inhibitors (NRTIs)
  2. Nonnucleoside reverse transcriptase inhibitors (NNRTIs)
  3. Protease inhibitors
  4. Fusion inhibitors
  5. CCR5 antagionists
  6. Integrase strand transfer inhibitors (INSTIs)
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29
Q

Some patients who receive therapy demonstrate unexpected clinical deterioration despite improved CD4 count and decreased viral load

A

Immune reconstitution inflammatory syndrome (IRIS)

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30
Q

Two subtypes of IRIS

A
  1. Paradoxic

2. Unmasking

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31
Q

Patient exhibit worsening symptoms or a new manifestation of a known infection

A

Paradoxic IRIS

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32
Q

Diseases that were not previously suspected become apparent shortly after initiation of HAART

A

Unmasking IRIS

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33
Q

Most common pathogens associated with IRIS

A
  1. Mycobacterium (tuberculous and nontuberculous)
  2. Cryptococcus
  3. Herpesviruses
  4. Hepatitis B and C viruses
  5. Human papillomavirus
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34
Q

True or False
IRIS has also been described in patients with non-HIV immune deficiency (solid-organ and stem cell transplant recipients)

A

True

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35
Q

AIDS-defining malignancies

A
  1. Kaposi sarcoma (HHV 8)
  2. Non-Hodgkin lymphoma (EBV)
  3. Invasive cervical cancer (HPV)
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36
Q

An angioproliferative disorder that causes lesions marked by spindle cell proliferation, neoangiogenesis, inflammation, and edema

A

Kaposi sarcoma

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37
Q

Four clinical variants of KS

A
  1. Classic KS
  2. Endemic KS
  3. Transplant- or immunosuppression-related KS
  4. AIDS-associated KS
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38
Q

First described in elderly men of Eastern Europe or Mediterranean descent

A

Classic KS

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39
Q

Lesions typically occurring on the upper and lower extremities

A

Classic KS

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40
Q

Recognized in black adults and children in Africa

A

Endemic KS

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41
Q

Most frequently affected sites in oral KS

A
  1. Hard palate
  2. Gingiva
  3. Tongue
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42
Q

Treatment for KS

A

Palliative

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43
Q

Specific indications for treatment of KS

A
  1. Cosmetically disfiguring lesions
  2. Symptomatic oral or visceral lesions
  3. Pain or edema associated with lymphadenopathy
  4. Extensive cutaneous disease
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44
Q

Local treatments for KS

A
  1. Alitretinoin topical gel
  2. Local irradiation
  3. Intralesional chemotherapy injection
  4. Cryotherapy
  5. Laser therapy
  6. Surgical excision
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45
Q

Cornerstone of treatment for AIDS-KS of all stages

A

HAART

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46
Q

Agents currently FDA approved for systemic treatment of KS

A
  1. Liposomal anthracyclines (doxurubicin and daunorubicin)
  2. Paclitaxel
  3. Interefon-alpha
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47
Q

Other commonly used agents for systemic treatment of KS

A
  1. Vinca alkaloids (vincristine, vinblastine, vinorelbine)
  2. Bleomycin
  3. Etoposide
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48
Q

Second behind KS as the most common cutaneous malignancy in HIV-positive population (1.8%)

A

BCC

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49
Q

True or False
CD4 count does not correlate with the incidence or severity of BCC but may play a more significant role in the development of cSCC

A

True

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50
Q

An additional risk factor for the development of cSCC

A

Use of voriconazole in the treatment of invasive fungal infections in TRs and in patients with HIV

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51
Q

Most common site of HNSCC in immunodeficient patients

A

Larynx—>oral cavity—>oropharynx

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52
Q

Cervical and anogenital cancer in immunodeficient patients is associated with

A

HPV types 16, 18, 31, and 45

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53
Q

Factors leading to increased rate of lymphoma in HIV-patients and TRs

A
  1. Virally induced immunosuppression
  2. Decreased immune surveillance
  3. Altered cytokine exposure
  4. Chronic antigenic stimulation
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54
Q

Most common type of lymphoma to develop in HIV-positive patients and TRs

A

EBV-associated NHL

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55
Q

WHO classification for AIDS-related lymphomas includes three categories

A
  1. Lymphomas that also occur in immunocompetent patients
  2. Lymphomas that occur specifically in HIV-positive patients
  3. Lymphomas that also occur in other immunodeficiency states
56
Q

Lymphomas that also occur in immunocompetent patients

A
  1. Burkitt
  2. Diffuse large Bcell
  3. Hodgkin
57
Q

Lymphomas that occur specifically in HIV-positive patients

A
  1. Primary effusion lymphoma
  2. Large B cell lymphoma arising in HHV8-associated multicentric Castleman disease
  3. Plasmablastic lymphoma
58
Q

True or False
Most AIDS-related NHLs are EBV-associated B-cell lymphomas such as Burkitt lymphoma, diffuse large B-cell lymphoma, and PBL

59
Q

Alters tumor suppressor gene (TP53) expression and protein regulation

60
Q

Second most likely cancer-related cause of mortality in the HIV-positive population, behind lung cancer

61
Q

Most common manifestation of CNS involvement in NHL

A

Leptomeningeal disease

62
Q

Clinical tool used to predict survival in NHL

A

International Prognostic Index

63
Q

Factors associated with poor prognosis in NHL

A
  1. Age over 60 years
  2. Advanced tumor stage
  3. Elevated serum lactate dehydrogenase
  4. Poor performance status
  5. More than one extranodal site of disease
64
Q

A complication of solid-organ and stem cell transplantation marked by EBV-driven abnormal lymphoproliferation

A

Posttransplantation lymphoproliferative disorder

65
Q

Is the second most common malignancy to develop in Trs, after cutaneous malignancy

A

Posttransplantation lymphoproliferative discorder

66
Q

True or False

Most PTLDs are NHL of the B-cell type

67
Q

True or False
PTLD develops in the setting of severe immunosuppression, which impairs the formation of a cytotoxic T-lymphocyte immune response to EBV

68
Q

Treatment of PTLD

A

Directed first toward decreasing immunosuppression regimens to allow an adequate T-cell response

69
Q

True or False
PTLD: patients who fail to respond may require systemic therapy such as combination chemotherapy, cytokine therapy, or anti-CD 20 therapy (rituximab)

70
Q

True or False
PTLD: surgery is reserved for management of local symptoms; upper airway obstruction caused by adenotonsillar hypertrophy

71
Q

Risk factors for the development of HL in TRs include

A
  1. Bone marrow transplantation

2. History of GVHD

72
Q

Most common NADM that occurs in patients with HIV

73
Q

Characteristic pathologic cells seen in HL

A

Reed-Sternberg cells

74
Q

The accepted standard of treatment for HIV-HL consist of

A

Combination chemotherapy and ART

75
Q

True or False
Salivary flow rates have been shown to be diminished in the setting of HIV or GVHD, which leads to increased incidence of dental caries and impaired deglutition

76
Q

Majority of salivary gland masses in HIV-positive patients are the result of a benign entity known as

A

Benign lymphoepithelial cyst (BLEC)

77
Q

True or False
BLECs have cyst walls lined by hyperplastic and metaplastic squamous epithelium, and they contain aggregates of lymphoid proliferation

78
Q

True of False

HIV-associated BLECs typically do not require parotidectomy

79
Q

True or False
Parotidectomy or enucleation of lesions may be considered in BLECs that undergo rapid size change, are disfiguring, or have significant pressure symptoms

80
Q

Occurs in the setting of HIV and is marked by salivary gland enlargement with circulating and visceral CD8 lymphocytosis

A

Diffuse Infiltrative Lymphocytosis Syndrome

81
Q

Second most common cause of cervical lymphadenopathy in HIV-positive patients after benign reactive lymphadenopathy

A

Extrapulmonary TB

82
Q

Most common site of extrapulmonary TB involvement in HIV-positive patients

A

Lymph nodes

83
Q

Most common pathogens in acute sinusitis (HIV)

A
  1. Streptococcus pneumoniae
  2. Haemophilus influenzae
  3. Moraxella catarrhalis
84
Q

Most common pathogens in chronic sinusitis (HIV)

A
  1. Staphylococcus
  2. Pseudomonas species
  3. Anaerobes
85
Q

Important pathogens in immunodeficiency-related sinusitis, because these organisms have the potential to cause invasive and oftentimes fatal disease

A
  1. Rhizopus

2. Mucor

86
Q

Most common fungal pathogen in invasive and noninvasive sinusitis for both immunosuppressed and immunocompromised patients

A

Aspergillus fumigatus

87
Q

Suspicious findings for IFS on MRI

A
  1. Obliteration or infiltration of periantral or orbital fat
  2. Inflammatory changes in the EOMs
  3. Leptomeningeal enhancement
88
Q

True or False
Fungal sinusitis can extend via thrombophlebitic or hematologic spread and thus may enter into the orbit or intracranial cavity without histologic evidence of mucosal invasion

89
Q

Aspergillus may allow extension of disease without evidence of bony destruction on imaging through

A

Angiocentric invasion pattern

90
Q

Goal of management of sinusitis in immunocompromised patients include

A
  1. Swift treatment of bacterial sinusitis

2. Early identification of fungal sinusitis or neoplasm

91
Q

Initial medical management of acute bacterial sinusitis consists of

A
  1. Broad-spectrum antibiotics
  2. Decongestants
  3. Saline irrigation
92
Q

Ideal treatment involves three components

A
  1. Systemic antifungal therapy
  2. Surgical debridement of infected tissue
  3. Restoration of immune function
93
Q

True or False
The pathogenesis of SBO is further facilitated by the breakdown of the local cutaneous barrier in the EAC secondary to dermatologic lesions and self-induced trauma as a result of pruritus

94
Q

Spreading to the skull base with resulting temporal bone osteomyelitis occurs via

A
  1. Fissures of Santorini

2. Tympanomastoid suture

95
Q

Risk factors for invasive aspergillosis of the temporal bone include

A
  1. Low CD4 count
  2. AIDS diagnosis
  3. Neutropenia
  4. Corticosteroid therapy
  5. Antineoplastic therapy
  6. Prolonged antibiotic therapy
96
Q

Otoscopy on temporal bone osteomyelitis

A

White debris or granulation tissue at the bony cartilaginous junction of the EAC

97
Q

More specific for osteomyelitis, and some suggest its use to monitor response to therapy

A

Gallium-67 citrate scintigraphy

98
Q

Very sensitive but relatively nonspecific test for detecting increased osteoblastic activity

A

Technectium-99 scintigraphy

99
Q

Treatment of choice in bacterial SBO (cure rates near 90%)

A

Fluoroquinolones

100
Q

Subcortical dementia marked by memory loss, apathy, and difficulty with reading and comprehension in the early stages

A

HIV-associated dementia

101
Q

True or False
HAD is thought to be caused by either repeated exposure to infected monocytes that cross the BBB or by autonomous viral production in the brain, which serves as a reservoir for the virus

102
Q

Most common sensory neuropathy associated with HIV

A

Distal sensory polyneuropathy (DSP)

103
Q

Characterized by symmetric polyneuropathy and axonal degeneration

A

Distal sensory polyneuropathy

104
Q

Most common cranial nerve manifestation in cryptococcal meningitis

A

Hearing loss

105
Q

Should be suspected in any HIV-infected patient who is seen with cochleovestibular complaints

A

Otosyphilis

106
Q

True or False

Hearing loss as a result of syphilis is usually bilateral, may be progressive or fluctuate, or may have a sudden onset

107
Q

True or False
The audiometric curve in otosyphilis often shows a low-frequency hearing loss in association with diminished speech discrimination scores

108
Q

The first priority in the evaluation of SNHL in a patient with HIV infection

A

Rule out potentially life-threatening disease

109
Q

Most common oral manifestation of HIV infection in adults and children

A

Candida infection

110
Q

3 most common forms of oral candidiasis

A
  1. Pseudomembranous candidiasis
  2. Erythematous candidiasis
  3. Angular cheilitis
111
Q

Presents as smooth white or cottage cheese-like plaques that can occur on any mucosal surface

A

Pseudomembranous candidiasis (thrush)

112
Q

It is distinguished from other white lesions of the oropharynx in that the white plaques can be rubbed off; when the plaque is wiped off, an erythematous base remains

A

Pseudomembranous candidiasis

113
Q

Marked by mild or moderately erythematous patches

A

Erythematous candidiasis

114
Q

Presents as tender and erythematous fissures and ulcers at the oral commissure

A

Angular cheilitis

115
Q

A white lesion with a corrugated and shaggy surface caused by EBV

A

Oral hairy leukoplakia

116
Q

True or False

OHL most frequently occurs on the lateral surface of the tongue

117
Q

Risk factors for the development of oral hairy leukoplakia

A
  1. Low CD4 count
  2. High viral load
  3. Presence of oral candidiasis
118
Q

OHL is diagnosed on biopsy by the presence of

A
  1. Hyperkeratosis
  2. Acanthosis
  3. Clear or “balloon” cells in the upper spinous cell layer with minimal inflammation
  4. Presence of EBV in the basal epithelial cells
119
Q

Most common manifestation of herpes simplex infection of the oral cavity

A

Herpes labialis

120
Q

A herpes labialis which is larger and more numerous, persistent longer and recur more frequently

A

Fever blisters

121
Q

Treatment of choice for acyclovir-resistant HSV

A

IV foscarnet

122
Q

3 types of aphthous ulcers

A
  1. Herpetiform ulcers
  2. Minor aphthous ulcers
  3. Major aphthous ulcers
123
Q

Type of aphthous ulcer: smaller than 0.2 mm in diameter and are self-limited

A

Herpetiform ulcers

124
Q

Type of aphthous ulcer: well circumscribed, painful ulcers less than 6mm in diameter with an erythematous halo; in the HIV-infected patient, ulcers frequently coalesce to form larger lesions that last about 2 weeks

A

Minor aphthous ulcers

125
Q

Type of aphthous ulcer: larger than 6 mm in diameter, are painful, persist for weeks, and threaten nutritional intake

A

Major aphthous ulcers (Sutton disease)

126
Q

Initial treatment of minor ulcers

A

Over the counter protective and analgesic medications

127
Q

Treatment for major or nonhealing minor lesions

A

Thalidomide (200mg/day)

128
Q

Periodontal disease associated with HIV infection is classified into:

A
  1. Linear gingival erythema
  2. Necrotizing periodontal disease
  3. Enhanced progression of chronic periodontal disease
129
Q

Presents with distinct linear erythema that does not respond to conventional periodontal therapy and is closely related to the presence of OC

A

Linear gingival eythema

130
Q

Presents with ulceration of the interdental papilla associated with gingival bleeding and pain that may invade the alveolar bone

A

Necrotizing periodontal disease

131
Q

Mainstay of therapy for HIV-related gingivitis and periodontitis

A
  1. Dental plaque removal

2. Oral rinses with 10% povidone-iodine with 0.1% to 0.2% chlorhexidine gluconate

132
Q

Refer to collectively to the changes in fat distribution that occur in HIV-positive patients who receive ART

A

HIV-associated lipodystrophy

133
Q

Changes (HIV-associated lipodystrophy)

A
  1. Peripheral lipoatrophy
  2. Lipohypertrophy
  3. Increased breast size and abdominal girth and increased visceral adipose tissue
  4. Metabolic disturbances (hypertriglyceridemia, hypercholesterolemia, insulin resistance, T2Dm, elevated hepatic transaminases)
134
Q

Fat distribution that affects the face and extremities

A

Peripheral lipoatrophy

135
Q

Manifest as increased fat deposition in dorsocervical fat pads (“buffalo hump”) or the lateral and anterior neck (“bullfrog neck”)

A

Lipohypertrophy

136
Q

Characterized by fat atrophy in the malar, buccal, melolabial, and temporal regions

137
Q

Current FDA-approved treatments for facial LA

A
  1. Calcium hydroxylapatite

2. Poly-L-lactic acid

GENERAL OTOLARYNGOLOGY (12 decks)

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