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1 Path Unit > 13 HIV Amyloidosis (starts at amyloid) > Flashcards

13 HIV Amyloidosis (starts at amyloid) Flashcards

1
Q

On standard light microscopy amyloid forms what on H&E?

A

amorphous pink deposits

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2
Q

The amyloid proteins form 7.5 to 10 nm fibrils arranged in alpha helix or beta sheets?

A

beta sheets

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3
Q

Does amyloid take up congo red? What happens when polarizing filters are applied?

A

Yes—conge red stain reveals apple-tree birefringence

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4
Q

What are the 3 types of insoluble fibrils in the chart on amyloid formation?

A

AL protein
AA protein
ATTR protein

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5
Q

What is the soluble precursor misfiled protein for AL protein?

A

Immunoglobulin light chains

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6
Q

What is the soluble precursor misfiled protein for AA protein?

A

SAA protein

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7
Q

What is the soluble precursor misfiled protein for ATTR protein?

A

Transthyretin

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8
Q

Which precursor of amyloid is due to production of normal amounts of mutant protein?

A

transthyretin

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9
Q

Which precursor of amyloid is formed by production of abnormal amounts of protein with chronic inflammation?

A

SAA protein

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10
Q

Which precursor of amyloid is formed by production of abnormal amounts of protein with an unknown or possibly carcinogenic stimulus?

A

Immunoglobulin light chains

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11
Q

Review the list of secondary causes of amyloidosis-

A
  1. Tuberculosis/Leprosy
  2. Chronic suppuratio
  3. Rheumatic Diseases
  4. Ulcerative colitis
  5. Lymphogranulomatous
  6. Sarcoidosis
  7. Regional ileitis
  8. Malignant tumors/especially kidneys
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12
Q

T-F–almost any organ can be effected with amyloid deposits?

A

True–remember pictures of heart, shin, flank, tongue, extremely dark black eye.

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13
Q

T-F– amyloidosis is a consideration in only 2 diagnostic differentials?

A 
False--multiple diagnostic differentials
[Review the List]
-Neuropathy, sensory
-nephrotic syndrome
-heart failure and arrhythmias
-malabsorption
-organomegaly
-carpal tunnel syndrome
-intracerebral hemorrhage
-bleeding disorders
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14
Q

What are the 6 classic primary immunodeficiency diseases we need to know?

A
  1. severe combined immunodeficiency SCID
  2. Immunodeficiency with Hyper IgM
  3. Common Variable Immunodeficiency CVID
  4. X linked agammaglobulinemia (Bruton’s)
  5. Selective IgA deficiency
  6. DiGeorge Syndrome
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15
Q

Infections that are unexpected, too frequent, too severe, or abnormally persistent suggest what?

A

immunodeficiency

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16
Q

Opportunistic infections in patients without known risk factors?

A

immunodeficiency

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17
Q

T-F –typical autoimmunity is a reason to suspect immunodeficiency?

A

False–atypical

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18
Q

What does unexplained lymphadenopathy or splenomegaly suggest?

A

immunodeficiency

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19
Q

Atypical presentations of hematopoietic malignancies suggest?

A

immunodeficiency

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20
Q

What type of inflammation and fever might suggest immunodeficiency?

A

recurrent unexplained

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21
Q

Does a family history of any factors of immunodeficiency suggest immunodeficiency in that person?

A

Yes [any factors suggesting immunodeficiency in combination with developmental abnormalities also makes it stronger]

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22
Q

Humoral immune deficiency has what 3 strong clinical characteristics?

A
  1. recurrent infections with extracellular encapsulated bacterial pathogens
  2. Recurrent sinopulmonary disease
  3. Few fungal or viral infections, giardiasis
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23
Q

T-F–humoral immune deficiency may or may not lack B cells?

A

True

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24
Q

T-F humoral immune deficiency usually has striking growth retardation?

A

False

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25
Q

What are 3 strong clinical characteristics of cellular immune deficiency?

A
  1. Recurrent infections with low grade opportunistic agents like fungi, virus or pneumocystis
  2. Near fatal reactions to live virus vaccines or BCG
  3. Susceptibility to graft-versus host disease
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26
Q

T-F– cellular immune deficiency is commonly accompanied by growth retardation, wasting, diarrhea?

A

True

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27
Q

T-F– cellular immune deficiency has an increased incidence of malignancy?

A

True

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28
Q

Are there tons of new immune deficiencies identified every year?

A

Yes…15 in the last 2 years

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29
Q

According to the 2009 IUIS update, how many categories of primary immune deficiencies are there?

A

8 [Just to review]

  • combined
  • defined syndromes
  • predominantly antibody
  • immune dysregulation
  • congenital defects of phagocytes
  • defects in innate immunity
  • autoinflammatory
  • complement
30
Q

What is the most common SCID severe combined immune deficiencies?

A

x linked recessive-50%

[autosomal recessive SCID is also a popular one]

31
Q

In x linked recessive SCID, what is the defect?

A

common gamma chain shared by a bunch of IL receptors

[IL-2,4,7,9,15]

32
Q

The absence of which IL receptor may be most critical in SCID> why?

A

IL-7- essential for the proliferation of lymphocyte precursors

33
Q

A deficiency in what protein which transducer signals from the common cytokine receptor gamma chain can also produce autosomal recessive SCID?

A

Janus Kinase 3 JAK3

34
Q

What is the most common cause of autosomal recessive SCID?

A

deficiency in adenosine deaminase

35
Q

In the less severe combined deficiencies, either due to the CD40L (XLR) and CD40 (AR) what is seen among the B-cells?

A

They are present. IgM is increased. IgG, IgA, and IgE are absent because there is not class switching.

36
Q

What type of infections are seen in Hyper IgM combined deficiencies?

A

Opportunistic infections, autoimmune cytopenias, gastrointestinal disease

37
Q

What are the 2 defined syndromes with immunodeficiency?

A

DiGeorge

Wiskott-Aldrich

38
Q

What syndrome with immunodeficiency is caused by defective thymic development? Which type of T cells are most depressed?

A

DiGeorges

CD8 cells are usually most depressed

39
Q

Severe for of Di georges clinical spectrum is embraced by what acronym? Where is the micro deletion?

A 
Catch 22  (22q11.1-22q11.2)
cardiac
abnormal faces
thymic hypoplasia
cleft palate
hypocalcemia
40
Q

What syndrome with deficiency is described as T cells progressively declining, IgM down and IgA and IgE are up?

A

Wiskott-Aldrich Syndrome

41
Q

What are the causes of death for wiskott-aldrich syndrome?

A

infection, hemorrhage and lymphoid neoplasms

42
Q

What type of response to polysaccharide antigens is found in wiskott-aldrich syndrome?

A

defective response

43
Q

What chromosome contains the wiskott-aldrich syndrome? what other syndrome is mutated on the same gene?

A

x chromosome

x-linked thrombocytopenia

44
Q

What are the cellular effects of the gene from wiskott-aldrich syndrome? Does the mutation in it exhibited phenotypic variation?

A

actin polymerization and signal transduction

yes, significant phenotypic variation

45
Q

A severe decrease in Ig and B cell numbers suggests which disease?

A

x linked recessive agammaglobulinemia (Brutons)

[tyrosine kinase mutation]

46
Q

A severe decrease in Ig and variable B cell numbers suggests what disease?

A

common variable immunodeficiency

47
Q

Selective immunoglobulin deficiency and normal B cells?

A

Selective IgA deficiency

48
Q

What are the 4 characteristics of Bruton’s agammaglobulinemia?

A
  1. B cells and Ig decreased
  2. Infections usually by 8-9 months, heterogenous
  3. Organisms: pyogenic bacteria, enterovirus, Giardia lamblia
  4. defect encodes cytoplasmic tyrosine kinase (btk)
49
Q

What type of bacterial infections happen in common variable immunodeficiency?

A

recurrent pyogenic [also, enterovirus, herpes, G lamblia]

50
Q

What type of deficiency do relatives of people with common variable immunodeficiency have? If you want to review…what are the mutations?

A

IgA deficiency

[mutations=TACL, BAFF-R, CD19, ICOS]

51
Q

What is the most common immunodeficiency in people of european origin? Most individuals are asymptomatic or symptomatic?

A

IgA

asymptomatic, some have bacterial infections

52
Q

What can occur in IgA deficiency people when exposed to IgA?

A

anaphylaxis

53
Q

T-F–there are familial associations between IgA deficiency and CVID?

A

True…mutations of both diseases are sometimes found in the TACI gene.

54
Q

What disease is defined by defect in Tcell apoptosis and deletion?

A

Autoimmune lymphoproliferative syndrome [can be autosomal dominant or recessive]

55
Q

What cellular effect is caused by autosomal recessive autoimmune polyendocrinopathy with candidiasis and ectodermal dystrophy APECED?

A

Defect in T cell deletion in the thymus

56
Q

What is the defect of IPEX immune dysregulation polyendocrinopathy enteropathy x linked?

A

defect of immunoregulatory T cells

57
Q

What disease is a defective respiratory burst with infections with organisms such as catalase positive staph

A

chronic granulomatous disease CGD

58
Q

What disorder has delayed cord separation, infection and leukocytosis?

A

LAD leukocyte adhesion deficiency

59
Q

What infections are commonly seen with gamma interferon disorders?

A

mycobacteria and salmonella

60
Q

Defects of innate immunity [that lead to bacterial, mycobacterial, viral, papillomavirus, and herpes simplex] are caused by defects/mutations in what 3 main areas?

A

-modulation of NFkB
-mutation of TLRs
-Mutation of chemokine receptors
[and the signaling pathways with these proteins]

61
Q

The disordered production of IL1B and its closely related antagonist IL1RN are associated with what kind of disorder ?[what are some of the symptoms for review]?

A

Autoinflammatory disorders
[recurrent fevers, serositis, cold urticaria, uneitis, hearing loss, arthritis, aseptic meningitis, osteomyelitis, inflammatory skin rashes and bowel disease, AMYLOIDOSIS]

62
Q

Early component complement deficiency (C1q and C1r and C4) lead to what diseases?

A

SLE-like syndrome

rheumatoid-like disease

63
Q

LAter component complement deficiency (C5, 6, 7, 8a, 8b, and 9) lead to what diseases

A

neisserial infections and SLE-like disorders

64
Q

C1 esterase inhibiotr deficiency leads to what disease?

A

angioedema

65
Q

CD55 and CD59 deficiency leads to what disease?

A

acquired paroxysmal nocturnal hemoglobinuria

66
Q

Ebstein barr virus is a common diagnosis reflex for what PID?

A

XLR lymphoproliferative disorder

67
Q

Papilloma and HSV is a common PID diagnostic reflex for what disorder?

A

gamma IFN

68
Q

opportunistic infections in infants is a sign of what PID diagnosis? older children?

A

SCID

CD40/CD40L

69
Q

Common bacterial repeated infections is a PID diagnostic reflex for what?

A

XLA and CVID

70
Q

REVIEW THE GENERAL PID DIAGNOSTIC REFLEXES

A 
autoimmunity
allergies/anaphylaxis
lymphadenopathy/splenomegaly
development abnormalities
graft vs. host
radiation sensitivity
*fever without infection
*inflammation without infection

1 Path Unit (16 decks)

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