Immunologic: entanercept or infliximab (TNF-a inhibitors)
How is SLE diagnosed?
ANA (antinuclear Ab)
Anti-dsDNA & anti-Sm MORE specific, LESS sensitive
Lupus band test: Ig & complement in dermal epidermal junction
What is the lupus band test?
Looks for Ig and & complement in dermal-epidermal junction
Describe the various lesions of scleroderma.
Streaks(linear) (en coup de sabre)
What is CREST syndrome?
Type of systemic slceroderma
Telangiectasias (dilated suface vessels)
What does schrirmer’s test measure?
Tera production (confirm sjogrens w/biopsy of salivary glands of lower lip
Drugs with negative log ratio are selective for?
What is Burton’s x-linked agammaglobulinemia?
Males w/o Ig’s
No mature B cells, germinal centers, plasma
Tx: Ig replacement
Defective bTK stops development from pre-B to immature B
Dx: NO CD19+ cells
What are the 3 ways Ig work?
3. Complement activation
What is isolated IgA def?
Intrinsic B cell defect (TH dystfunction, suppressor T cells) may have anti-IgA Abs
Asymptomatic, may have some sickness
Other Ig levels are normal
What is often associated w/ IgG subclass deficiency?
Tx: IV IgG
What is Hyper IgM def?
X linked mutation in CD40L-> high IgM low other Ig
Pyogenic bacteria infections
Dx: hypercellular node w/ no distinguishable germinal center
What is DiGeorge syndrome?
3rd and 4th branchial arch, no thymus, no mature T Cells -> no active B cells
ears/eyes lower, eyes spaced apart
What happens in the thymus?
T cells mature: cortex=education
What is CVID?
Umbrealla for impared B cells
High infection, autoimmune
What is SCID?
Severe combined immunodeficiency
Variable forms most commonly x-linked (IL-2 receptor)
Defective T and B cell function
Tx: antimicrobial & BMT
What does a mutated IL-2 receptor cause?
No activation of T cells (gamma chain)
What does a SCID thymus look like?
No germinal centers, no clonal expansion, different architecture
What is Wiskott-Aldrich?
X linked: eczema, bacterial infections thrombo-cytopenia (WASp)
Decreased T cell, defective NK-cells, increased autoimmune/cancers
WASp expressed in lymphocytes and megakaryocytes
What is CGD?
Defect in monocyte and neutrophil respiratory burst (H2O2, O2, NO, myeloperoxidase)
Cannot kill CATALASE+ bacteria
What is the respiratory burst?
NADPH + 2 O2 -> NADP+ 2O- -> SOD-> H2O2
What does myeloperoxidase do?
Creates chloramines (bleach)
What is hereditary angioneurotic Edema?
HAE C1 defect (lacks inhibition)
Prevented immune complex formation
Alternative pathway: C3
Classical pathway: C1qrs, C4 C2
Terminal Complement: C5-9
Chemo regimen of induction, consolidation and maintenance/conditioning.
Induction: 1st cycle, decrease the number of malignant cells.
Consolidation – once the cells are no longer detectable another cycle is given to further reduce the ones that are not seen. (once the numbers get below 5% of the cells seen on a smear/biopsy, then it is considered undetectable)
Maintenance – done to keep the cells undetectable
What is non-myeloablative?
Chemo then TBI(radation) on day of transplant-> mild pancytopenia, delays GVHD
What is critical to prevent infections for oral mucositis?
Depressed immune, open sores, different oral flora
How are Fl trays used?
Daily, 1-3 drops, 4-10 minutes at night when less salivary flow