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32 Cards in this Set
- Front
- Back
what is caused by deficiency of C1 esterase inhibitor, with low serum C4 and consumption of complement components like C3?
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hereditary angioedema
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what is the hypersensitivity reaction: target cells are killed by Fc receptor bound cytotoxic leukocytes, no complement involvement, and major NK cell involvement.
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Antibody-dependent cell-mediated cytotoxicity (ADCC)
subtype of type II |
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which disease?
type III HS rxn; deposit antigen-antibody complexes in many sites like heart, joints, kidneys |
serum sickness
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what HS type is SLE?
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type III
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what HS type is arthus reaction?
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type III
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what HS type is polyarteritis nodosa?
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type III, involves small and medium sized arteries
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name the disease:
- X linked, present in male infants, found at 6 mos - no Ab synthesis due to failure of B cells to mature - no plasma cells or immunoglobulins - recurrent bacterial infections |
Bruton's agammaglobulinemia
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disease?
- occasional anaphylactic rxn to transfused blood and infections, especially in mucosal surfaces |
isolated IgA deficiency
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disease?
- congenital T-cell deficiency - abnormalities of mandible, ear, aortic arch - recurrent viral and fungal infections - tetany |
DiGeorge syndrome
CATCH 22: Cardiac defects Abnormal facies Thymic hypoplasia Cleft palate Hypocalcemia microdeletion of chromosome 22 |
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disease?
- low B and T cell, severe defects in humoral and cell-mediated immunity - adenosine deaminase deficiency (ADA), leading to toxins against lymphocytes - failure to thrive |
SCID: severe combined immunodeficiency disease
(aka Swiss-type agammaglobulinemia) |
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immunodeficiency disease?
- eczema, thrombocytopenia and recurrent infection - x-linked disorder with normal total immunoglobulins - poor Ab response to polysaccharide antigens |
Wiskott Aldrich syndrome
TIE: Thrombocytopenia Infections recur Eczema |
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how does HIV infection begin?`
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HIV virion expresses a cell surface protein, gp120, with binding sites for the CD4 molecule on those T cells
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which collagen disease?
- Raynaud phenomenon - serosal inflammation (pericarditis and pleuritis) - diffuse interstitial pulmonary fibrosis - atypical non bacterial endocarditis - immune complex vasculitis |
SLE
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which collagen disease?
- ANA reacts to Sm (Smith) antigen - biologic false positive for syphilis |
SLE
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disease?
- ANA anti-Scl-70 - anti centromere - widespread fibrosis and degenerative change |
CREST syndrome (scleroderma)
Calcinosis Raynaud phenomenon Esophageal dysfunction Sclerodactyly Telangectasia |
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disease?
- hypertrophy of collagen fibers of subcutaneous tissue -> fixed facial appearance - sclerodactyly - dysphagia - interstitial pulmonary fibrosis - HTN |
scleroderma
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which collage disease?
triad: 1. dry mouth (xerostomia) 2. keratoconjunctivitis 3. occurs with other connective tissue or autoimmune disease - involves salivary glands |
Sjogren syndrome
anti-SS-B antibodies are found |
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which collagen disease?
- xerostomia (dry mouth) + keratoconjunctivitis |
Sicca syndrome (Sjogren syndrome)
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which collagen disease?
- chronic inflammation of proximal muscles of extremities - increased serum creatinine kinase - biopsy shows necrotic muscle cells and lymphcytes |
polymyositis
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disease?
- women 35-40 yrs - arthralgia, Raynaud phenomenon, esophageal hypomotility - ANA anti-nRNP, with speckled nuclear appearance |
MCTD: mixed connective tissue disease
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disease?
- found in men - abdominal pain, HTN, uremia, polyneuritis, splenomegaly, fever etc. - immune complex vasculitis with segmental fibrinoid necrosis in walls of small and medium sized vessels |
polyarteritis nodosa
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what type of amyloidosis is involved in multiple myeloma and in Waldenstroms macroglobulinemia?
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primary amyloidosis, caused by deposition of AL (amyloid light chain) protein
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what amyloid disease is a complication of chronic inflammatory disease, including RA, TB, osteomyelitis, syphilis or leprosy?
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secondary (or reactive systemic) amyloidosis
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disease?
- amyloid derived from transthyretin - severe peripheral nerve involvement due to amyloid deposits |
Portuguese type of polyneuropathy
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disease?
- A4 or amyloid-beta protein deposits - the code for this protein is on chromosome 21 |
Alzheimer disease
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disease?
- episodic fever and polyserositis - similar distribution and type to that of secondary (AA) amyloidosis - characteristic demographic |
familial Mediterranean fever
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disease?
- prominent amyloid deposits in the tumor derived from calcitonin |
medullary carcinoma of the thyroid
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disease?
- amyloid deposit in islet cells - amyloid is known as islet amyloid polypeptide (IAPP) |
diabetes mellitus (type 2, insulin resistant)
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disease?
- minor deposits of amyloid found at autopsy in the elderly - when occurring in the heart, the amyloid is derived from transthyretin |
senile amyloidosis
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disease?
- amyloid deposits in joints of pts who have undergone years of dialysis - amyloid derived from beta-microglobulin |
dialysis-associated amyloidosis
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what is the CREST syndrome?
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a scleroderma subtype:
Calcinosis Raynaud's phenomenon Esophageal dysfunction Sclerodactyly Telangectasia |
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what is the CATCH 22 in DiGeorge syndrome (aka thymic hypoplasia)?
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CATCH 22:
Cardiac defects Abnormal facies Thymic hypoplasia Cleft palate Hypocalcemia microdeletion of chromosome 22 |