BRS: Immune Dysfunction
Terms
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- what is caused by deficiency of C1 esterase inhibitor, with low serum C4 and consumption of complement components like C3?
- hereditary angioedema
- what is the hypersensitivity reaction: target cells are killed by Fc receptor bound cytotoxic leukocytes, no complement involvement, and major NK cell involvement.
-
Antibody-dependent cell-mediated cytotoxicity (ADCC)
subtype of type II -
which disease?
type III HS rxn; deposit antigen-antibody complexes in many sites like heart, joints, kidneys - serum sickness
- what HS type is SLE?
- type III
- what HS type is arthus reaction?
- type III
- what HS type is polyarteritis nodosa?
- type III, involves small and medium sized arteries
-
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
-
disease?
- occasional anaphylactic rxn to transfused blood and infections, especially in mucosal surfaces - isolated IgA deficiency
-
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 -
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) -
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 - how does HIV infection begin?`
- HIV virion expresses a cell surface protein, gp120, with binding sites for the CD4 molecule on those T cells
-
which collagen disease?
- Raynaud phenomenon
- serosal inflammation (pericarditis and pleuritis)
- diffuse interstitial pulmonary fibrosis
- atypical non bacterial endocarditis
- immune complex vasculitis - SLE
-
which collagen disease?
- ANA reacts to Sm (Smith) antigen
- biologic false positive for syphilis - SLE
-
disease?
- ANA anti-Scl-70
- anti centromere
- widespread fibrosis and degenerative change -
CREST syndrome (scleroderma)
Calcinosis
Raynaud phenomenon
Esophageal dysfunction
Sclerodactyly
Telangectasia -
disease?
- hypertrophy of collagen fibers of subcutaneous tissue -> fixed facial appearance
- sclerodactyly
- dysphagia
- interstitial pulmonary fibrosis
- HTN - scleroderma
-
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 -
which collagen disease?
- xerostomia (dry mouth) + keratoconjunctivitis - Sicca syndrome (Sjogren syndrome)
-
which collagen disease?
- chronic inflammation of proximal muscles of extremities
- increased serum creatinine kinase
- biopsy shows necrotic muscle cells and lymphcytes - polymyositis
-
disease?
- women 35-40 yrs
- arthralgia, Raynaud phenomenon, esophageal hypomotility
- ANA anti-nRNP, with speckled nuclear appearance - MCTD: mixed connective tissue disease
-
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
- what type of amyloidosis is involved in multiple myeloma and in Waldenstroms macroglobulinemia?
- primary amyloidosis, caused by deposition of AL (amyloid light chain) protein
- what amyloid disease is a complication of chronic inflammatory disease, including RA, TB, osteomyelitis, syphilis or leprosy?
- secondary (or reactive systemic) amyloidosis
-
disease?
- amyloid derived from transthyretin
- severe peripheral nerve involvement due to amyloid deposits - Portuguese type of polyneuropathy
-
disease?
- A4 or amyloid-beta protein deposits
- the code for this protein is on chromosome 21 - Alzheimer disease
-
disease?
- episodic fever and polyserositis
- similar distribution and type to that of secondary (AA) amyloidosis
- characteristic demographic - familial Mediterranean fever
-
disease?
- prominent amyloid deposits in the tumor derived from calcitonin - medullary carcinoma of the thyroid
-
disease?
- amyloid deposit in islet cells
- amyloid is known as islet amyloid polypeptide (IAPP) - diabetes mellitus (type 2, insulin resistant)
-
disease?
- minor deposits of amyloid found at autopsy in the elderly
- when occurring in the heart, the amyloid is derived from transthyretin - senile amyloidosis
-
disease?
- amyloid deposits in joints of pts who have undergone years of dialysis
- amyloid derived from beta-microglobulin - dialysis-associated amyloidosis
- what is the CREST syndrome?
-
a scleroderma subtype:
Calcinosis
Raynaud's phenomenon
Esophageal dysfunction
Sclerodactyly
Telangectasia - what is the CATCH 22 in DiGeorge syndrome (aka thymic hypoplasia)?
-
CATCH 22:
Cardiac defects
Abnormal facies
Thymic hypoplasia
Cleft palate
Hypocalcemia
microdeletion of chromosome 22