8. Acquired and Genetic Disorders of the Lymphatic Vascular System Flashcards

1
Q

Lymphatic System Function

• Drainage of interstitial fluid from body tissues and return to bloodstream
• ____ and ____ immune response
– ____ (B, T, and NK cells)
– ____ cells (monocytes, macrophages, neutrophils, etc)

A

humoral
cell-mediated
lymphocytes
supporting cells

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2
Q
Lymphatic System Structure
• Closely associated with the \_\_\_\_ system
• \_\_\_\_ to prevent backflow (like veins)
• Components: 
– Lymph nodes
– Lymphatic vessels 
– Lymph
A

cardiovascular

valves

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3
Q
Lymph 
Composition
• \_\_\_\_ cells
• \_\_\_\_ proteins
• Extra interstitial fluid
• Can also transport \_\_\_\_ cancer cells

Named for lympha, the Roman deity of ____

• Metastatic cancer can also travel via \_\_\_\_
A

white blood
large
metastic
fresh water

blood

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4
Q

Lymphoid Structures in H&N

• Lymphoid structures within the oral cavity
	○ Waldeyer's ring
		§ \_\_\_\_ tonsils
		§ \_\_\_\_ airways
A

asymmetric

compromised

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5
Q

Lymphatic System Disorders
• ____
– Abnormal collection of interstitial fluid & fibroadipose tissue

• ____
– Abnormal lymph node enlargement

• Lymphoma and metastatic cancers

A

lymphedema

lymphadenopathy

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6
Q

Lymphedema Overview

  • Primary lymphedema: lymphedema due to ____ etiologies
  • Secondary lymphedema: lymphedema ____ to acquired conditions
	• Secondary
		○ \_\_\_\_, cancer, infection, etc.
	• Malformation in drainage in one of the fingers
		○ Primary lymphedema
		○ Resolve by surgery
A

congenital/inherited
secondary
surgical

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7
Q

Primary Lymphedema
• Primarily affects the ____ extremities, but may be found ____ the body
• May progress ____ or resolve ____ throughout development
• Classified by ____ at onset

A
lower
throughout
slowly
spontaneously
age
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8
Q

Malformations of Vessels
• Abnormality resulting in ____ of interstitial fluid
○ Lymphatic malformation

[NOTES]

A

accumulation

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9
Q

Primary Lymphedema
• Clinical presentation reflects area affected, for example:

Tongue, mouth, trachea
____, ____, speech disturbance, difficult ____

Orbits
____, diplopia

Bone
Bone ____ or overgrowth

Skin
____, pain, swelling

Chest
____, wheezing, ____

A

dyspnea
dysphagia
feeding

proptosis

hypertrophy

cellulitis

chest pain/pressure
dyspnea

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10
Q

Primary Lymphedema
• Lymphatic Malformations
– Rare ____ deformities of the lymphatic system due to abnormal development of the lymphatic vasculature

• Drainage in the lower extremities, resulting in \_\_\_\_ swelling
• Cystic hygroma
	○ \_\_\_\_ that can be resected
A

congenital
unilateral
cystic structure

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11
Q

Primary Lymphedema
May occur as part of a syndrome including ____ syndrome

	• \_\_\_\_ of X
	• Presents in \_\_\_\_
	• Webbing of the \_\_\_\_
		○ Primary lymphedema
	• \_\_\_\_ corrected
A
turner
monosomy
females
neck
surgically
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12
Q

Secondary Lymphedema
• Etiologies include ____, trauma, ____, inflammatory disorders, cancer and cancer treatments

* More \_\_\_\_
* Primary lymphedemas are rare
A

infection
burns
common

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13
Q

Secondary Lymphedema
SVC Syndrome

  • Obstruction of the superior vena cava (SVC) due to thrombosis or compression by pathologic process in adjacent structure
  • Associated with ____ and ____ etiologies
  • Symptoms include ____ and marked interstitial ____ of the head and neck• Swelling of neck and face; shortness of breath; lungs, coughs, shortness of breath and dysphagia
    • Large ____ mass
    ○ Lymphoma
    ○ Tumors from ____
    ○ ____ tumors
    • Compression of vessels that drain HN region
    • Can also be caused by thrombosis (bloot clot)
    ○ No smooth flow: area is prone to thrombosis
    • Blood clot and compression results in significant swelling
A

benign
malignant
dysphagia
lymphedema

medistinal
thymus
germ cell

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14
Q

Secondary Lymphedema
Post-surgical Lymphedema

  • Most common etiology is ____ lymph node dissection for ____ cancer
  • Also seen in patients following ____ and/or radiation• ____ lymph node biopsy
    ○ Dye injected in area of tumor, and see where it’s draining (radioactive dye)
    ○ Ultimately resecting the lymph node where the drain is flowing
    ○ Complication: ____
    § If axillary lymph nodes are all removed: significant swelling
    • Compression sleeves allows lymph to recirculation
    • OT lymph massages
    • Life-long complication
    • Treating the lymphedemic arm is ____ (where the dissection was)
    ○ Do not use the arm for drawing ____, etc
    • ____ can mimic the phenotype of lymphedema
    ○ ____swelling
A

axillary
breast
chemotherapy

sentinel
lymphedema
limited
blood
thrombosis (DVD)
unilateral
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15
Q

____ Lymphedema
Lymphatic Filariasis (Elephantiasis)
• ____infection of lymphatics and subcutaneous tissue by filarial worms transmitted by ____
• Can cause disfiguring swelling resulting in pain, disability, and social stigma
• Epidemiology: Over 140 million people infected worldwide

• Common in \_\_\_\_ countries
A

secondary
parasitic
mosquitoes
tropical

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16
Q

____ Lymphedema
Obesity

• Would be \_\_\_\_
A

secondary

bilateral

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17
Q

Lymphadenopathy Overview
• ____: widespread lymph node enlargement
• ____: lymph node enlargement in one region

A

generalized

localized

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18
Q

Lymphadenopathy
• Etiologies include ____, autoimmune conditions (e.g. ____), drug side effects, and ____

• Of note, lymphadenopathy may be ____ or entirely ____

A

infections
sarcoidosis
cancer

painful
asymptomatic

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19
Q

Lymphadenopathy
• A “normal” lymph node is about ____ cm – about the size of a fingernail, or pea
– May be slightly larger in ____

A

1

adolescents

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20
Q

Generalized Lymphadenopathy

• Often associated with ____ disease

A

systemic

21
Q

____ Lymphadenopathy
Infectious Mononucleosis
“the ____ disease”

* \_\_\_\_ symptoms
* Test for \_\_\_\_
* \_\_\_\_ can be enlarged
A
generalized
kissing
"B"
EBV
spleen
22
Q

Generalized Lymphadenopathy
Infectious Mononucleosis (cont.)
• Classic triad of symptoms: ____, ____, ____
– Some patients also present with persistent fatigue, palatal petechiae, hepatosplenomegaly, jaundice, or tonsillar exudate

• 90% of cases associated with Epstein-Barr virus (____ herpesvirus transmitted via ____)
• Reactive lymphocytes demonstrate ____-like morphology (____ cells)
– Hence, mononucleosis

• 70-80% of humans experience EBV at some point in their lifetime
A

fever
pharyngitis
cervical lymphadenopathy

ubiquitous
saliva
monocyte
Downey

23
Q

Generalized Lymphadenopathy
Human Immunodeficiency Virus (HIV)
• Retroviral infection characterized by reduced ____ resulting in progressive immunodeficiency

A

CD4+ Tcells

24
Q

Generalized Lymphadenopathy
HIV (cont.)

• ____ infection
– Fever, lymphadenopathy, sore throat, rash, joint or muscle pain, and
headache
– Some patients are ____

• Chronic infection
– Many patients are asymptomatic, or present with persistent
generalized ____
– Symptomatic chronic infection includes ____, oral hairy leukoplakia, ____, peripheral neuropathy, and idiopathic thrombocytopenia ____

• Test every patient for \_\_\_\_, may not necessarily be a lymphoma
A

acute
asymptomatic

lymphadenopathy
thrush
herpes zoster
purpura

25
Q

Generalized Lymphadenopathy

Mycobacterium
• Tuberculosis mycobacterium
– M. tuberculosis, bovis, and africanum; cause
tuberculosis (TB)
– 95% of TB patients present with \_\_\_\_

• Nontuberculous mycobacterium
– Usually restricted to ____ infection
– Cause of 90% of mycobacterium-associated ____

A

cervical lymphadenopathy
localized
cervical lymphadenitis

26
Q

Localized Lymphadenopathy

• Etiologies include local infections/reactions, ____, and ____

A

implants

cancers

27
Q

____Lymphadenopathy

Streptococcal Pharyngitis
• Symptoms: 
– Sore throat
– \_\_\_\_
– Tender cervical adenopathy 
– \_\_\_\_
• Symptoms respond to \_\_\_\_ therapy; usually resolve within \_\_\_\_ days
• Etiology: infection by \_\_\_\_bacteria
A
tonsillar exudate
fever
antibiotic
2-5
group A streptococcus (GAS)
28
Q

Localized Lymphadenopathy
Cat Scratch Disease
• Infection by ____ transmitted by cat scratch/bite or flea bite
• Local infection in up to 90% of cases
– Regional ____ and/or acute cutaneous inflammatory
reaction near site of bite/scratch
• Bloodborne dissemination may cause life-threatening ____ and ____ complications or ____ involvement
• About 22,000 cases in the United States annually
– Most common among ____ and ____

A
bartonella henselae
lymphadenopathy
visceral
neurologic
ocular
immunocompetent
young adults
29
Q

Localized Lymphadenopathy
Cat Scratch Disease (cont.)

  • ____ lesion on finger
  • Enlarged ____
  • Ocular involvement (____ star), rare
A

papular
lymph node
macular

30
Q

Lymphoma
Risk Factors
• Causes are unknown for most people

– Genetic
• Unclear
• No strong ____ patterns (studies in
identical twins)

– Environmental factors
• Some chemicals suspected (pesticides/herbicides/____)
• High-dose ____ exposure

– Immuno-suppression
• Post–organ ____, autoimmune diseases, HIV

– Viral and bacterial infections
• ____ virus, EBV, ____, Hepatitis C

	• HTLV-1
		○ Caribbean and japan
		○ T cell \_\_\_\_
	• HepC
		○ Removing the HepC may cause the lymphoma to enter \_\_\_\_
A

familial
benzenes
radiation

transplant
HTLV-1
H. pylori

lymphoma
remission

31
Q

Lymphoma
• ____ group of blood cancers arising from lymphocytes
• Malignant lymphocytes usually accumulate within lymph nodes forming ____ appearing masses
• Some extra-nodal forms may infiltrate skin, bone marrow, GI tract, lung, or peripheral blood

	• Can infiltrate the \_\_\_\_
		○ Bleeding
	• \_\_\_\_ biopsy
		○ Needle in enlarged lymph node
		○ Cylindrical piece
	• Prefer remove \_\_\_\_
		○ Grade the lymphoma, determine severity
A
heterogenous
solid
gums
needle
whole lymph node
32
Q

Lymphoma
Symptoms
• B symptoms: systemic symptoms characteristic of lymphomas
– ____
– ____
– ____ (10%)
• Other characteristic symptoms include:
– Painless lymphadenopathy (may be observed during ____)
– ____

A
fevers
night sweats
uninended weight loss
dental exam
splenomegaly
33
Q
Lymphoma Work-up
• HPI and physical exam
• Labs
– Complete blood count
– Metabolic panel
– \_\_\_\_ (NHL)
– \_\_\_\_ rate (HL)
• Radiographic imaging studies
• Tissue biopsy
– Lymph node and/or bone marrow
	• Staging
		○ Observing where the disease is
	• PET-CT
		○ Activity within the structures
		○ Injected with \_\_\_\_
			§ Areas of metabolic activity
			§ Yellow = metabolically \_\_\_\_
	• A residual mass would not pick up any of the \_\_\_\_
	• PET-MRI
		○ No \_\_\_\_ whatsoever
A

lactate dehydrogenase
sedimentation

FDG (radiolabeled glucose)
active
FDG
radiation

34
Q
Hodgkin Lymphoma: Overview
• 8,500 cases in the U.S. annually
• \_\_\_\_  of all lymphomas
• Common in \_\_\_\_ patients
• High \_\_\_\_  rates
• Growing number of survivors (over 150,000 in U.S.)

• Hodgkin lymphomas are ____ lymphomas
• ____ cells
○ Malignant cells in hodgkin’s

A

10%
young
cure

B-cell

Reed-Sternberg

35
Q

Non-Hodgkin Lymphoma
• Very ____ group (over 50 subtypes)
• Incidence ____ with age
• Accurate pathology is ____

A

heterogenous
increases
essential

36
Q

Non-Hodgkin Lymphoma
Clinical Classification

	• Three subtypes:
		○ \_\_\_\_
		○ \_\_\_\_
		○ \_\_\_\_
	• Depends on behavior of lymphoma within the patient
A

indolent
aggressive
highly aggressive

37
Q

Non-Hodgkin Lymphoma
Indolent
• ____ growing - may take ____ to progress
• Often ____, usually ____
• May not require treatment initially or ____ antibodies only
• Respond well to therapy, but ultimately ____(“non-curable”)

	• Small, enlarged lymph nodes
		○ Arm pits, etc
	• \_\_\_\_ waiting:
		○ No proof that treating patients early is of any benefit
	• When to treat:
		○ Large lymph nodes
		○ Symptoms
			§ Mechanically affecting the patient
		○ Cosmetic
	• The theory of \_\_\_\_ won't necessarily work because they target rapidly dividing cells
		○ Use of \_\_\_\_ instead
A
slow
years
asymptomatic
advanced
monoclonal
relapse

watchful
chemotherapy
monoclonal antibodies

38
Q

Non-Hodgkin Lymphoma
Aggressive
• ____ growing - ____ to develop
• Often associated with ____ symptoms (fevers, night sweats, weight loss)
• ____ (with or without radiation therapy) at diagnosis
• Most common: ____ has cure rate over 60% in advanced disease
• Can be cured at relapse with ____
• ____ (low grade) lymphomas can transform to high grade (aggressive) disease

A
fast
months
B
chemotherapy
DLBCL
stem cell transplant
indolent
39
Q

Non-Hodgkin Lymphoma
Highly Aggressive

• ____ progression/cell doubling time
• May present as oncologic emergency (spontaneous tumor ____, cord compression,
obstruction)
• High ____ at diagnosis, but respond well to chemotherapy and curable (up to 80%)
• Require CNS ____, inpatient chemotherapies

	• \_\_\_\_ lymphoma
		○ Jaw tumors
		○ HIV-related
	• \_\_\_\_ dividing tumor in human body
		○ Very sensitive to chemotherapy
	• 20% of those uncured:
		○ Spontanteous tumor lysis
		○ \_\_\_\_ failure due to K+ levels (and arrythemias)
	• As long as you treat properly highly curative (80%)
A

rapid
lysis
mortality
prophylaxis

burkitt’s
fastest
renal

40
Q

Lymphoma
Treatment
• Important Factors for Consideration

– \_\_\_\_ of lymphoma
– Stage
– Prognostic characteristics
– \_\_\_\_
– \_\_\_\_ of treatment
– Other health issues (“co-morbidities”)
• Treatment depends on \_\_\_\_ and health
• Treatment is currently not tailored to patients
	○ \_\_\_\_
		§ ABVD
• Future: individualized treatment
	○ Send tumor for molecular signature and determine the best course of action
A

subtype
symptoms
toxicity profile

age
chemo-cocktails

41
Q
Lymphoma
Chemotherapy
-Short term side effects
- Fatigue
• Nausea
• Low blood counts (risk of neutropenic fevers, bleeding)
• Hair \_\_\_\_
• Neuropathy
• Cognitive changes: “\_\_\_\_”
• Constipation/diarrhea
• \_\_\_\_ ulcers
  • Long term side effects
  • Organ impairment (lungs, heart, fertility, bone marrow, peripheral nerves)
  • Secondary malignancies (leukemia, another lymphoma, solid organ cancers)
	• Side effects in mouth are common
		○ Lining of \_\_\_\_
		○ Cells with high turnover
	• Mucositis
		○ Difficulty eating, swallowing
	• "\_\_\_\_"
		○ Viscous lidocaine
		○ Soothing liquid
	• Immuno-suppressed
		○ \_\_\_\_-related issues in mouth
		○ \_\_\_\_ thrush
A

loss
chemo-brain
mouth

mouth
magic mouthwash
HSV
oral

42
Q

Lymphoma
Monoclonal Antibodies

“Most of the B-cell lymphoma have expression of ____ and
____ works on attacking the cells in different ways and mobilizing the immune system to work on lymphoma.”

A

CD20

rituxan

43
Q
Lymphoma
Targeted Therapies
• Radioimmunotherapy (RIT)
– \_\_\_\_antibody labeled with a \_\_\_\_ delivers targeted
radiation directly to the tumor site
• "\_\_\_\_"
	○ Goes to area with\_\_\_\_-positive B cells
	○ Delivers radiation directly to the tumor in small amounts
A

monoclonal
radionuclide
liquid radiation
CD20

44
Q

Lymphoma
Cellular Therapies
• Stem Cell Transplantation – Autologous stem cells
– Allogeneic stem cells

  • Chimeric Antigen Receptor modified T-cells (CAR T cells)
  • Genetically modified, ____ (patient’s own) ____ specifically directed to attack tumor cells
A

autologous

Tcells

45
Q

Targeting Tumors with Chimeric Antigen Receptors
Native TCR

  • CARs combine an recognition piece of an antibody with signaling (activating) domain into ____ protein.
  • Gene transfer to stably express CAR on T cells confers novel Ag specificity.• Express receptors on T-cell that can attach to CD-19 on tumor cells:
    ○ Specifically target the tumor cells
    • Remove T-cells;
    • Modify them and use a ____to induce change in the DNA of the T-cells;
    • Now begins to express receptors;
    • And introduce the modified T-cells back into the patient
    • CART
    ○ ____ storm
    § T-cells are ____ and cause inflammatory issues (fevers, ICU)
A

single chimeric
viral vector
cytokine release
overactive

46
Q
Oral Complications of Cancer Treatment
• Oral mucositis
• Alteredtaste
• Salivary gland dysfunction
• Deferred/delayed dental care
• Osteonecrosis of the jaw
• Radiation caries
• Oral mucositis as a result of both \_\_\_\_ and \_\_\_\_
• SC transplant
	○ Auto: patient's own SC are modified (high dose of chemo killing last cancer cell)
	○ Allogenic: transplant from \_\_\_\_else
		§ Various immune issues: graft vs host disease
			□ Dry mouth, lesions within mouth (sickle syndrome…?)
• Anti-mimetics/anti-nausea medicine
• ONJ
	○ Due to \_\_\_\_ used in solid tumors (bone metastases)
A

chemotherapy
radiation
someone
bisphosphonates

47
Q

Summary
• Disorders of the lymphatic system commonly involve impaired immune function or abnormal lymphatic vasculature
• ____ and ____ are often associated with both acquired and congenital disorders

A

lymphedema

lymphadenopathy

48
Q

Summary, cont.
• Genetic/congenital disorders often manifest at ____ or during ____
• Acquired disorders include ____ and ____ etiologies
• Malignancies like lymphoma may present as ____ or ____disease
– Complications involving the head and neck may be ____ or due to ____ itself

A
birth
puberty
infectious
non-infectious
nodal
extra-nodal
treatment-related
malignancy