Exam 3: Section 4 Continued Flashcards
Primary immune deficiency disorders
Genetic
Early onset (6-24 months)
Recurrent infections
Bruton Disease aka
X-linked agammaglobulinemia
Bruton Disease affects
Males, 6 months old
Bruton disease
B cells can’t mature, which means
No antibodies
Bruton disease can become more apparent after recurrent ___ and ___
Bacterial and viral infections
Treatment for Bruton Disease
IgG infusions
Good prognosis
Severe Combined Immunodeficiency (SCID) symptoms
Lymphopenia and lymphoid atrophy
SCID occurs in
1 in 65,000 (native Americans)
1 in 100,000 (other groups)
____ immune deficiency disorders more common than ___
Secondary
Primary
Secondary immune deficiency disorders can occur from
Infections
Malnutrition
Cancer
Therapy- induced immunosuppressive (MC)
Mechanisms of secondary immune deficiency disorders
- Bone marrow dysfunction
2. Lymphocyte dysfunction
Example of lymphocyte dysfunction
Acquired immunodeficiency syndrome (AIDS)
AIDS spread in
Blood
Semen
Vaginal fluid
Breast milk
AIDS destroys
CD4 T cells
Macrophages
Dendritic cells
Most common AIDS transmission in US
Male -male sex
48%
Declining
Most common AIDS transmission in Africa and Asia
Male-female
34%
Increasing
17% of AIDS spread via
IV drug abusers
<1% of AIDS spread via
Blood recipients
<1% AIDS spread via
Transplacental
Childbirth
Milk
> 80% of cases of AIDS are ____ transmitted
Sexually
HIV Diagnosis
- ELISA test
2. Western blot
3 Phases of HIV/AIDS
- Acute
- Chronic
- Crisis
Acute Phase of HIV
3-6 weeks post infection experience “flu-like” symptoms
High viremia
Death of many CD4 T cells
Viremia of AIDS decreases around
12 weeks
Chronic phase of HIV
2-10 years
Generalized lymphadenopathy, fatigue, fever
Steady decrease of CD4 T cells
Minor infections
Minor infections that can occur in chronic stage of HIV
Candidiasis
HSV
Crisis Phase of HIV
<200 cells/microliter
Viremia spikes
Cachexia
Fatigue
Fever >1 month
HIV binds to ___ on CD4 T cells
Gp120
HIV undergoes conformational changes due to interactions with
CCR5 protein
HIV crosses BBB by infecting
Monocytes - macrophages
Opportunistic infections
- Pneumocystis Pneumonia
- Candidiasis
- Cytomegalovirus
- TB
Tumors common in HIV
- Kaposi Sarcoma
2. Lymphoma
Amyloidosis occurs when extracellular fibrillar proteins are deposited and cause
Tissue damage and dysfunction
Amyloid deposits are composed of
Non branching fibrils
Amyloid deposited are unable to be removed by
Phagocytosis
Forms of amyloid proteins
- AL
- AA
- A beta
- TTR
AL proteins are associated with
Multiple myeloma
AA proteins are deposited in patients with
Chronic inflammatory conditions
Beta amyloid deposited in
Alzheimer’s disease
Transthyretin (TTR) is a normal protein that accumulates in excessive amounts as a form of
Amyloidosis
Older patients are most likely to have TTR accumulate following
Mutations in protein misfolding
When TTR accumulates in the walls of the heart, it is called
Senile cardiac amyloidosis
When TTR accumulates in multiple organ systems, it is called
Senile systemic amyloidosis
Patterns of amyloid depositions
- Systemic
2. Localized
MC cause of systemic amyloid distribution
AL proteins accumulated after multiple myeloma
Systemic amyloid distribution has a ___ prognosis
Poorer
Localized amyloid distribution causes changes in ___ and ___ in a singular region of the body
Tissue structure and function
Common areas for localized amyloid distribution
Heat Lungs Brain Skin Liver Ligaments of wrist Tongue Larynx Kidney Spleen
Diagnosis of amyloidosis requires ___ evaluation
Histological
Congo red dye combined with polarized light produces
Apple-green birefringence
Characteristic of amyloidosis
____ and ___ are MC cause of death related to amyloidosis
Renal and heart failure
SCID Involved impaired development of
Lymphocytes (T and B cells)
Common symptoms of SCID within first year
- Oral candidiasis
- Severe diaper rash
- Cytomegaloviral infections
- Fungal infections of lungs
- Failure to thrive
- Varicella zoster viral infection
