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Pathophysiology - Intro > Immune Deficiency > Flashcards

Immune Deficiency Flashcards

1
Q

What is Acquired immunodefiency syndrome (AIDS)

A

disease of the immune system characterized by a reduction in the number of helper T cells (= more likely to get infections and some cancers)
progressive impairement of immune system by HIV (affects many organ system and is life threatening)

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2
Q

human immunodeficiency virus (HIV)
infects almost _________ people—nearly ___ people
each hour—with the development of AIDS being
a possible eventual outcome of HIV infection.

A

7,000 daily

300 hrly

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3
Q

How many ppl did AIDS kill in 2011?

A

1.7 million

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4
Q

How many ppl have AIDS or HIV currently

A

34 mill ppl, of which 68% live in sub saharan africa

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5
Q

Since the epidemic began 30 years ago, more
than ___ million people have contracted HIV and
nearly __million have died of AIDS.

A

60 HIV
30 from AIDS

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6
Q

Risk factors for HIV or AIDS

A

unprotected sex, multiple sex partners, having another sexually transmitted infection, IV drug use, being uncircumcised and being born to an infected mother

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7
Q

4 stages of HIV

A

Stage 1: infection stage, lasts few weeks w/ short flulike illness
Stage 2: clinically asymptomatic stage, 10 yrs long, can still transmit
Stage 3: symptomatic HIV such as diarrhea, fever or night sweats, fatigue or joint pain, oral infections, enlarged lymph nodes, skin problems
Stage 4: Transition from HIV to AIDS (T cell count less tan 200)

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8
Q

What is the cause of AIDS?

A

HUMAN IMMUNODEFICIENCY VIRUS (HIV)

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9
Q

how is hiv transmitted?

A

contaminated body fluids (blood, vaginal secretions, semen, breast milk)

during sex, birth, breastfeeding, and sharing needles

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10
Q

how is HIV diagnosed?

A

using ELISA to detect HIV antibodies which are usually produced within 3 months of being infected and is confirmed with a Western blot
- ELISA results r often negative during 1st month of infection althought result of p24 antigens or polymeerase chain reaction (PCR) assay may be positive
- Rapid HIV antibody testing has become preferred method of testing

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11
Q

What is the recommended treatment for HIV ?

A

antiretroviral therapy (ART) which controls the replication of the virus and slows the progression of HIV-related diseases
- periodoc measurement of number of CD4 T cells and amt of HIV RNA present in blood (both markers of disease progression)

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12
Q

what drugs does ART include?

A

reverse transcriptase inhibitors - interferes with the conversion of HIV RNA to DNA

protease inhibitors - stops the assembly of new HIV viruses

fusion inhibitors - prevent the fusion of HIV to helper T cells

entry inhibitors - blocks HIV from entering helper T cell

integrase inhibitors - interfere with HIV by inserting its genetic material into human cells.

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13
Q

The World
Health Organization (WHO) and the Joint United
Nations Programme on HIV/AIDS (UNAIDS) estimate that at least ___ million people worldwide
were in need of ART therapy in 2010

A

15

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14
Q

WHO suggestions to prevent HIV

A

Abstinence
• Monogamy
• Condom use
• Testing and counseling for HIV
• Male circumcision
• Sterile needles and syringes for each injection for IV drug use
• ART therapy
➢ Preexposure prophylaxis for an HIV-negative partner
➢ Post-exposure prophylaxis for accidental exposure
➢ To prevent mother-to-child transmission

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15
Q

Cause of immunodeficiency disorders?

A
  • depressed or absent immune response
  • primary = decrease in no of t cells and b cells, leaving body unable to defend itself against infection and tumors
  • secondary = disease, infection, aging, damage to immune system frm drugs, radiation or surgery
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16
Q

What is immunodeficiency?

A
  • can occur in B cells, T cells, complement or pagocytes
  • can be in humoral (antibody related) or cell-mediated responses
  • individual doesnt have capability to dispose of foreign and harmful substances
  • incrased susceptibiity to bacterial = B-cell deficiency, viral, fungal and protozoan infection = T-cell deficiency
  • can be genetic and present at birth or can manifest later at like
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17
Q

How does acquired immunoincompetence result from?

A
  • bacterial or viral insult to body
  • malnutrition
  • radiation
  • certain drugs
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18
Q

what occurs as clinical course of AIDS progreses?

A
  • LYPHADENOPATHY
  • weight loss
  • fatigue
  • diarrhea
  • night sweats
  • frequent infection as number of T cells lowers (esp opportunistic infections, pneumonia, fever and malignancies)
  • in later stages, encephalopathy and malignancy lead to dementia and death
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19
Q

malignnacies and common opportunistic Infections and conditions in pt w/ aids

A
  • Kaposi sarcona
  • lyphomas
  • pneumocystis carinii penumonia (PCP)
  • tuberculosis
  • Herpes simplex infection
  • shingles
  • candida albicans
  • toxoplasmosis
  • neurologic complications like inflammation of nerves, neuropathy, neoplasms and AIDS dementia complex
  • diarrhea
  • epstein-barr virus
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20
Q

etiology of AIDS

A
  • caused by HIV, type 1 or 2 (type 1 = worldwide, 2 = W africa)
  • retroviruses that contain RNA, cant survive apart from human cells
  • destruction of T cells and proliferation of HIV leaves body denseless by reducing cell-mediated immunity
  • directly damages nervous system
  • soread by direct contact w/ blood or semen (sexual contact is primary means)
  • most cases occur w/ men having sex with men (MSM) in US and worldwide it’s heterosexual transmission
  • can be transmitted to infants of infected mothers through placenta, birth process and from breast milk
  • sharing needles
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21
Q

What is HAART?

A

Highly active antiretroviral therapy consists of a 3 drug combo:
- nucleoside reverse transcriptase inhibitor (NRTI) and either a non-nucleoside reverse transcriptase inhibitor (NNRTI) or protease inhibitor (PI)

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22
Q

What is drug has a 92% reduction in risk of HIV infection?

A
  • Preexposure prophylaxis (PrEP)
  • 2 meds in pill: tenofovir and emtricitabine (labeled under brand Truvada)
23
Q

What is common variable immunodeficiency (another name, what does it increase risk for)?

A
  • aka hypogamma gobulinemia
  • acquired B-cell deficiency that results in decerrase antibody prodcution and/or function
  • increased risk for development of autoimmuine disease and lymphoma
  • pt often succumb to chronic lung disease or malignancies
  • lower levels of Ig and B cells r associated w/ poorer prognosis
24
Q

s/s of common variable immunodeficiency (CVID)

A
  • history of chronic or recurrent infection like pneumonia, bronchitis, sinusitis, and otitis media
  • GI disease is often seen with such symptoms as diarrhea, abd pain and wt loss
  • lymphadenopathy
  • splenomegaly
  • hepatomegaly
  • Chronic lung disease and granulomatous disease
  • increased frequency of autoimmune disorders like autoimmune hemolytic anemia or immune thrombocytopenia
25
Q

etology of CVID?

A
  • 2 peaks of incidence: 1 btwn 18 and 25, smaller peak btwn 1 and 5
  • exact cause is not known, thought to be resuy=lt of genetic defects leading to immune system dysregulation and failure of B-cell differntiation
  • number of circulating B cells is often normal but ability of B cells to produce all types of Ig is reduced
  • some pt have impaired T-cell signaling
  • cell-mediated immnity usually remains intact
26
Q

How to diagnose and treat CVID?

A

diagnose - history of repeated and chronic infections leads to further investigation of Ig levels and T-cell quantities, antibody response to vaccines is poor, hypogammaglobulinemia and IgA and/or IgM deficiency r indicative

treatment - aimed to prevent infections and implement early treatment with appropriate antibiotic administration when infections occur, regular Ig replacement, antiotic usage and hospitalizations

27
Q

What is selective immunoglobulin A deficiency?

A
  • fail to produce normal levels of IgA (selective eficiencies of IgM or IgG have been report but r rare)
28
Q

s/s of selective immunoglobulin A deficiency

A
  • majority of pt w/ IgA deficiency r asymptomatic, perhaps bc of compensatory increase in IgM production in these pt
  • some other s/s r recurrent sinopulmonary infections, GI infections and/or autoimmune disease, food allergies, anaphylactic reactions to blood transfusions may occur, recurrent otitis media, sinusitis and pneumonia
29
Q

etiology of selective immunoglobulin a deficiency

A
  • failure of b cells to produce adequate levels of IgA is most common immunologic defect
  • most common risk for disease is family history of either IgA deficeicny or CVID
30
Q

how to diagnose and treat selective immunoglobulin a deficiency?

A

diagnose - pt who r older than 6 months and have recurrent sinopulmonary infections should have IgA levels measured, if below-normal levels of circulating IgA levels, CBC, complement assay and vaccine response testing r used to rule out other immunodeficiency, diagnosis is not definite until after age 4 years

treatment - no cure, treatment is geared toward prevention and management of infection, prophylactic antibiotics may be given, IV immune globulin w/ low concentrations of IgA may be tried

31
Q

What is x-linked agammaglobulinemia?

A
  • aka Bruton agammagobulinemia
  • condition chracterized by near absnece of serum immunoglobulins and icnreased susceptibillity to infection
32
Q

s/s of x-linked agammaglobulinemia?

A
  • absent or near-absent tonsils and adenoids
  • lymphadenopathy and splenomegaly r also noticeably missing
  • susceptible to recurrent severe gram-positive infections including otitis media, bronchitis, pneumonia, osteomyelitis, and meningitis
  • infection w/ enteroviruses r laos common
  • first noted btwn 3 and 18 months
33
Q

etiology of Bruton agammaglobulinemia?

A
  • congenital X-linked disorder affects only males
  • cauwsed by defect in Bruton tyrosine kinase (BTK) gene which is normally expressed in B cells uring all stages of development
  • all 5 Ig classes r usually absent, along w/ absence of circulating B cells and presence of normal numbers of circulating T cells
  • increased infection w/ encapsulated bacteria and certain viruses
  • most affected individuals present w/ symptoms by 5 years
  • around 40% of pt have an affected fmaily member
34
Q

how to diagnose and treat bruton agammaglobulinemia?

A

dinagose - thorough history including family history of relatives who died of severe ifnection, immunoelectrophoresis (indicates decreased levels of serum IgM, IgA, and IgG, however not valid before infant is 6-8 months of age), normal t-cell w/ low or absent b-cell, assess BTK gene mutation

treatment - IV infusions or subcutaneous administrations of immune globulin every 2 to 4 weeks + antiobtics (never live virus vaccines, corticosteroids or immunosuppressive drugs)

35
Q

What is severe combined immunodeficiency?

A

SCID
- result from disturbance in development and function of T cells with or without B cells
- bc t-cell signaling is needed for B cells to produce antiboidy, this leads to absence of both cell-mediated (T cell) immunity and antibody-mediated (B-cell) immunity, NK cells may or may not be present
- referred to by some as Bubble Boy disease
- etiolgy is x-linked or autsomal recessive (different genetics mutatiosn that leads to defect in stem cells differntiation into B cells and T cells = disruption in lymphocyre development)

36
Q

s/s of SCID?

A
  • severe, recurrent infections w/ bacteria, viruses, fungi, and protozoa
  • chronic diarrhea
  • failure to thrive
  • occurs by 3-6 months when natural maternal palcental immuinty begins t obe deplted
  • common infections r PCP and persistent mucocutaneous candidiasis
  • discernible lymphoid tissue may be absent
  • abnormal lab findings often include hypogammaglobulinemia and very low or absnet T cells
37
Q

diagnosis and treatment of SCID?

A

diagnose - now part of routine newborn screening, lab tests for measurment of Ig levels, antibody titers, number of T nd B cells (Lymphopenia w/ less than 20% of lymphocytes being T cells), test T-cell response, absnece of thymic shadow on chest radiography

treat - BMT is only cure, gene therapy is being investigated (stem cells to be impaltned in bone marrow using busulfan to make space for stem cells to grow, show to have children develop normal immune systems), supporttive therapy including IVIG and prophylaxis for Pneumocystis jiirovecii and respiratory syncytial virus (RSV), completely sterile environment

38
Q

What is Digeorge anomaly ?

A

thymic hypoplasia or aplasia
- congential condition of immunodeficiency that results from defective development of pharyngeal pouch system and presents w/ cardiac anomalies, hpoplastic thymus, hypocalcemia

39
Q

s/s of DiGeorge syndrome?

A
  • often ahve structural abnormalties
  • abnormally wide-set, downward slanting eyes
  • low-set ears with notched pinnae
  • small mouth
  • abnormalities of palate
  • CV defects like tetralogy of Fallot
  • thymus and parathyroid glands are absent or underdeveloped
  • signs of tetany resulting from hypocalcemia caused by hypoparathyroidism
  • some degree of cogntive impairement
  • susceptibel to infctions such as sinopulmoanry infectiosn and thrush
40
Q

etiology of DiGeorge anomaly?

A
  • result of abn development of 3rd and 4th pharyngeal puches during 12th wk of gestation
  • causes thymus glands to be underdeveloped or absent, cardiac defects and parathyroid hypoplasia
  • leads to defects in T-cell number function and in some cases decreased B-cell function as well
  • some of these children will not be clinically immunodeficent, others will ahve increased sinopulmonary infections and up to 1% will ahve life-threatening SCID as result of absent thymus
41
Q

how to diagnose DiGeorge syndrome?

A

diagnose - reduced number of T cells, 2 of these features (CV defect, hypocalceimia of greater 3 week duration, microdeletion involving chromosome 22, detected y fluorsecen in situ hybridization), absence, hypoplasia or abn placement of thymus gland can be detected radiographically

42
Q

how to treat DiGeorge syndrome?

A
  • treat hypocalcemia to stabilize myocardium and reduce risk of seizures
  • vtamin D and parthyroid hormone replacement therapy
  • repair of cardiac anomalies
  • thymic transplantation or BNT may reconstitute immune function in pt w/ most severe from fo DiGeorge
  • appropriate developmental interventions
  • saferty of live vaccines in case by ccase
43
Q

prognosis DiGeorge anomaly?

A
  • complete DiGeorge anomaly is usually fatal within 1st yr of life bc cardiac dysfunction, hypocalcemia and/or infection
  • partial DiGeorge anomaly, prognosis depends on severity of cardiac and parathyroid defects
  • immune function however may imporve with age and should be monitored regularly
44
Q

What is chronic mucocutaneous candidiasis?

A
  • refers to group of disorders characterized by persistent and rcurrent candidal fungal infectiosn of skin, nails and mucous membranes
  • unified by iclusion of deficient cell-meidated immunity to Candida organisms
45
Q

s/s of chronic mucocutaneous candidiasis

A
  • usually develop durign 1st 2 or 3 yrs of life, or if late-onset, in young adulthood
  • large circular lesions appear on skin, mucous membranes, nails, or vagina
  • sores in mouth amke eating difficult
  • reccuring thrush or diaper rash is often 1 symptom in infants
  • late stages expereine recurring respiraotry tract infection
  • associated endocrine disorders or autoimmune conditions
  • later onsent CMC can be associated w/ myasthenia gravis, thyoma, or bone marrow anbnormalites
  • systemic Candida infections r not seen in these pt
46
Q

What are syndromes of CMC?

A
  1. chronic oral candidiasis (affects mucosa of lips, tongue and buccal cavity)
  2. CMC w/ endocrinopathy (hypoadrenalism, hypothyroidism, hypoparathyroidism, and ovarian failure in females)
  3. chronic localized candidiasis (cutaneous lesions w/ hyperkeratosis)
  4. chronic diffuse candidiasis 9widespread infection of skin, nails and mucous mebmranes)
  5. candidiasis with thyoma (candidiasis accompanied by hypogammaglobulinemia, neutropenia, aplastic anemia, or myasthenia gravis)
47
Q

etiology of CMC?

A
  • in immunocompetent individual, Candida albicans is a nonpathogenic normal flora
  • pt w/ CMC have a T-cell deficit specific to Candida that makes them susceptible to infection
  • mutations in autoimmune regulator gene (AIRE) or STAT1 gene have been impliated in CMC pathogenesis
48
Q

how to diagnose CMC?

A

diagnose - history of recurrent Candida infections, potassium hydroxide preparations of scrapings from elsion show yeast pseudohyphae (string of atypical fungal cells), fungal cultre grows Candida, blood studies indicate normal T-cell circulation and normal antibody response to all organism expect Candida (normal Ig levels, lymphocte nu,bers and normal response to non-Candida antigens)

49
Q

how to treat CMC?

A
  • directed at eliminating the infections along with correcting the immunologic defects
  • systemic therapy w/ antifungal agents is the mainstay of treatment
  • chronic use of antifungals may prevent recurrence
  • transfer factor of protein of T lymphocytes isolated from donors sensitized t oCandida has been shown to increase immune response to Candida in pt w/ CMC
  • topical treatment w/ antifungal agents appears to improve skin and nail lesions
50
Q

What is Wiskott-aldrich syndrome?

A
  • congenital disorder that is characterized by inadequate B and T cell function, thrombocytopenia and eczema
51
Q

s/s of Wiskott-aldrich syndrome

A
  • eczema
  • thrombocytopenia with sever bleeding
  • increased susceptibility to bacterial and viral infections
  • bleedign manifestations iclude petechiae, purpura, hematemesis, epistaxis, hematuria and more serious AGI and intracranial bleeding
  • in 50% of pt w/ Wiskott-Aldrich sundrome,eczema develops in first yr of life
  • child r predisposed to development of autoimmune disorders (hemolytic anemia, vasculitis, etc.) and lymphoma
52
Q

etiolgoy of Wiskott-Aldrich sydnrome?

A
  • rare immunodeficiency disorder is inherited as X-linked trait affecting only males
  • arises from mutations in gene encoding the Wiskott-Aldrich syndrome protein (WASP) which is expressed in hematopoietic cells
  • thymus gland is normal at birth but decreases in size a child grows up, resulting in decreased B- and T- cell functions
  • metabolic defects in paltelet synthesis that cause platelets to be short lived and phagocyte to be incompetent r both present
  • avg age of diagnosis is 21 months
53
Q

Diagnosis and treatment of Wiskott-Aldrich sydnrome?

A

diagnosis - boy w/ thrombocytopenia nad small platelet size, esp when accompanied by eczema and frequent infection, diagnosis is supported by finding mutation is WAS gene, numer of T cells and B cells may be low, vaccine response may be defective, regultory t-cell function is impaired

treatment - BMT is only curative therapy, high succes s rate can be expected when donor is human leukocyre antigen (HLA)- matched sibling (if bmt is not feasible then splenectomy may result in icnreae in platelet number and size, additioanl treatnemtn consists of IVIG and appropriate prphylactic antibiotic therapy, topical system steroids r used to treat eczema, tirals of genere therapy r currently underway

54
Q

prognosis of Wiskott-Aldrich sydnrome?

A
  • long term survival is good for pt who receive BMT before 5
  • pt who undergo splenectomy w/o BMT have media survival of 25 years
  • those w/o splenectomy and BNT often die before age 5
  • bleeding, infections, and malignancy r main cause of deatu

Pathophysiology - Intro (15 decks)

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