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36 Cards in this Set
- Front
- Back
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Difference between patterns of acute versus chronic inflammation in serum protein electrophoresis.
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gamma globulins are increased in chronic inflammation.
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Negative acute phase proteins (3).
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prealbumin, albumin, transferrin.
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DIfference between pattern of renal versus GI protein loss on serum protein electrophoresis?
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Renal: alpha-2-macroglobulin and beta-lipoprotein may be increased (both are large, maybe compensatory increase in production). GI: smaller relative (or no) increase in a2m, beta-lipoprotein rarely increased.
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Major cause of anodal slurring of albumin band in serum protein electrophoresis.
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Hyperbilirubinemia (may also be caused by binding of drugs such as penicillin or heparin).
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Major features of liver disease in serum protein electrophoresis.
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Increased immunoglobulins, beta-gamma bridging (relative increase of IgA), possible anodal slurring of albumin (binding of bilirubin).
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Common cause of beta-1 doublet in serum protein electrophoresis?
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A polymorphism in transferrin results in a protein with slightly different migration pattern.
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Intravascular versus ex-vivo hemolysis on serum protein electrophoresis.
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Intravascular: free hemoglobin binds haptoglobin resulting in clearance, seen as a depressed alpha-2 region. Ex-vivo: free hemoglobin binds haptoglobin, increasing its cathodal migration (see an extra band between a2 and b1).
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Genetic defect in hereditary angioedema
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inherited deficiency or dysfunction of the C1 inhibitor
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Define the most common biochemical categories of acquired amyloidosis.
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AL (Ig light chain), AA (acute phase reactant), TTR (Transthyretin, "senile cardiac amyloidosis"), B2m (beta 2 macroblobulin (Lihgt chain component of MHC; associated with dialysis)
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Coagulation abnormality associated with amyloidosis? In what type of amyloidosis does it occur?
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Factor X deficiency is associated with AL amyloidosis (Ig light chains).
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Hepatitis B surface antigen is occasionally associated with which vasculitis
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PAN
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What are the three types of cyroglobulins
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Type I: monoclonal Ig's made by a neoplasm like myeloma or lymphoplasmacytic lymphoma. Type II: monoclonal IgM, plus a polyclonal IgG. Type III: combination of two polyclonal Ig's. Types II and III are the "mixed cryoglobulins," and are often caused by HCV infection.
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What is chronic granulomatous disease
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An x-linked defect in NADPH oxidase causing poor oxidative killing by monocytes. Associated with McLeod syndrome.
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What is Chediak-Higashi disease
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AR syndrome of oculocutaneous albisnism, neutropenia, and thrombocytopenia. Platelets and neutrophils have huge granules that cannot degranulate properly
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Histological diagnosis of Dermatitis herpetiformis
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Demonstrate IgA deposits along the nonaffected subepidermal basement membrane. Similar to celiac disease, antibodies against tissue transglutaminase (anti-tTG) are elevated in patients with the disease. Responds to gluten free diet.
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Diseases associated with anti-RNP antibodies
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mixed connective tissue disorder (high titer) or SLE (modest titer)
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Diseases associated with anti-dsDNA and anti-Sm antibodies
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essentially restricted to SLE
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Diseases associated with anti-Ro and anti-La antibodies
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Sjogren's syndrome
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Diseases associated with anti-Scl-70 antibodies
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Progressive systemic sclerosis
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Diseases associated with anti-Centromere antibodies
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CREST syndrome (also some progressive systemic sclerosis and Reynaud's)
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Diseases associated with anti-histone (H2A and H2B) antibodies
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Drug-induced lupus; typically also ANA positive, negative for dsDNA, Sm, RNP
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Pattern of ANCA reactivity in Wegener's granulomatosis?
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C-ANCA (cytoplasmic) is seen in ~90% of cases and is 95-99% specific. C-ANCA antibodies are anti-proteinase 3 (PR3). Titers correlate with disease activity.
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P-ANCA: target and diseases
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targets myeloperoxidase (MPO); seen in microscopic polyangiitis, polyarterisit nodosum, primary sclerosing colangitis, ulcerative colitis
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diseases associated with HLA-DR3
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IDDM, SLE, Sjogren's, myasthenia gravis, celiac sprue, Grave's
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diseases associated with HLA-DR4
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IDDM, RA, pemphigus vulgaris
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diseases associated with HLA-DR2
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MS, narcolepsy, protective for IDDM
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diseases associated with HLA-B27
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ankylosing spondylitis; In addition to its connection with AS, HLA-B27 is implicated in Reiter's syndrome, certain eye disorders such as acute anterior uveitis and iritis, psoriatic arthritis and Crohn's disease. Reiter's syndrome then again, is statistically associated with AS.
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Components of alpha-1 band in SPE
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alpha-1 antitrypsin (major component), a1-acid glycoprotein
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Components of alpha-2 band in SPE
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a2-macroglobulin, ceruloplasmin, haptoglobin
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Components of beta band in SPE
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transferrin, complement (C3, decreased with incubation at RT), beta-lipoprotein, b2-microglobulin. Fibrinogen present in beta-gamma interface if clot is incomplete.
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Components of gamma band in SPE
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immunoglobulins, C-reactive protein, lysozyme
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ANA: pattern and disease association
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The speckled pattern is most strongly associated with Mixed Connective Tissue Disease (MCTD)
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ANA: pattern and disease association
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The nucleolar pattern is associated with Progressive Systemic Sclerosis (PSS)
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ANA: pattern and disease association
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Anticentromere staining is associated with the CREST syndrome, a subgroup of PSS.
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What is myeloperoxidase deficiency
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AR defect in MPO enzyme. Patients get bacterial infections but are not hugely immunocompromised.
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ANA: pattern and disease association
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The diffuse staining pattern is the commonest, and the most strongly associated with SLE: most patients will have this. You can tell it's diffuse and not finely speckled because mitotic figures stain (would be negative in speckled).
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