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46 Cards in this Set
- Front
- Back
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Multiple Meyloma
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BENZ JONES proteins
M (MONOCLONAL) PROTEINS puched out lesions on X ray due to OSTEOCLASTS causing HYPERCALCEMIA. HYPERVISCOSITY SYNDROME - Px present with mucosal bleeding. marrow contains plasma cells. excessive IgG |
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Waldenstrom's Macroglobulinemia
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NO BENZ JONES PROTEINS
NO PUNCHED OUT LESIONS You have excessive IgM and EXCESSIVE PLASMA IN BONE MARROW Hyperviscosity due to IgM |
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MGUS
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No BENZ JONES
No Punched out lesions presence of M PROTEINS in serum. VERY LOW PLASMA LEVELS |
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Chediak Higashi Syndrome
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Mutation in LYST protein, impairs phagolysosome formation.
Cant swallow the bugger to begin with, but it has an endosome in tact and the radicals but cant get with the lysosomes. albinims MASSIVE GRANULES abnormal NK activity. Death by 5-10 yrs. |
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Chronic Granulomatous Disease
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Is going to have NADPH oxidase DEFICIENCY
GP91 phox If test (NBT) is purple, then NADPH oxidase exists. Growing granulomas can cause obstructive effects. Px can get infections from NORMAL FLORA. |
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Myeloperoxidase Deficiency
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No Myeloperoxidase to break down peroxide to make superoxide?
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Leukocyte Adhesion Deficiency
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Difficulty for neutro/eos/NK etc. to EXTRAVASATE.
Severe bacterial infections DELAYED WOUND HEALING deficiency in leukocyte integrin proteins. omphalitis |
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Hereditary Angioedema
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Deficiency in C1 INHIBITOR
Px has severe EDEMA C2 KININ is involved with EDEMA |
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Paroxyzmal Nocturnal Hemoglobnuria
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Mutated GPI anchor for DAF/MIRL/HRF/CD59
Inhibits C3b and C4b convertase so no amplification. Death via hemorrhage. RED CELL LYSIS** |
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Bruton's Hypogammaglobulinemia
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No B cells.
defect in CD79, thus no signal. Important for pre B cell in bone marrow to IMMATURE B CELL. mutation in Bruton TYROSINE KINASE. upper respiratory, NO TONSILS, normal T Cells NO B CELLS, NO PLASMA, NO IGs TX w/ PASSIVE ANTIBODIES with gamma globulin. |
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X linked hyper IgM syndrome
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Excessive IgM
Mutation in CD40 (145) no isotype switching thus low IgA,G,E |
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Selective IgA deficiency
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weak MUCOSAL defenses
you have IgA but you CANNOT SECRETE THEM. most common immunodeficiency disorder. DANGER OF ANAPHYLACTIC SHOCK DURING TRANSFUSION b/c 40% px make antibodies to IgA. Px are usual ASYMPTOMATIC. |
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Transient hypogammaglobulinemia of infancy
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Child has no IgG which it was getting from its mother.
It has other Immunoglobulins due to GammaDelta T cells. Found in premature infants. May require antibiotics |
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DiGeorge Syndrome
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22q11 gene
Hypothyroidism, hypoplasia of thymus & PTH. CATCH 22 NO T Cells esp in periphery. NO cell mediated adaptive response Has B Cells |
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Bare Lymphocyte I
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CD8 Deficiency
Defect in TAP I/II gammadelta T cells can help with viral infections to lessen effect of this SCID. |
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Bare Lymphocyte II
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mutation in transcription factors for MHC II thus no CD4.
No B-T Tango, no memory or effector cells. SECONDARY HYPOGAMMAGLOBULINEMIA. CIITA and RFX genes. Low IgM,A,G |
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Gamma Common Chain
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Thymic shadow.
Deficiency in IL 7/9/15/2/4 NO T Cells No Nk cells Has B Cells EXTREMELY LOW IgG normal IgM, IgA No antibodies to Tetaunus/Diptheria. |
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Wiskott Aldrich Syndrome
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No T Cells
Has B and NK cells Eczema and Thrombocytopenia LOW & ABNORMAL PLATELET COUNTS - DIAGNOSTIC Xp11.23, defect in WASP protein in MEGAKARYOCYTES. Begin with high level of T cells but decline. Recurring infections in Flu and Pneumonia. IgG - normal IgM - decreased IgE/A - increased As T cells decline poor B cell response due to lack of TANGO. Affects microtubules and actin thus lower amounts of cytokine reach their receptors. PARACORTICAL DEPLETION OF T CELLS. |
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Omenn Syndrome
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No T cell
No B cell Has Nk cell Thymic Shadow No IgA or M ELEVATED IgE**** |
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Reticular Dysgenesis
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No CD34, Thus DEFECT IN STEM CELLS.
NO MYELOID/LYMPHOID cells NEED BONE MARROW TRANSPLANT MOST SEVERE FORM OF SCIDS |
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ADA/PNP
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Once again a stem cell defects
ADA = No T or B cells due to high toxic metabolites. PNP = Declining T cells. for both LOW IMMUNOGLOBULIN LEVELS. lack 5' nucleotidase. |
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Kostmann Syndrome
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deficiency in ELA2 on neutrophils. Premature DESTRUCTION of MYELOCYTES.
TX with GM-CSF or bone marrow. (omphalitis, septicimia, absecess) 70% mortality, bacterial and cancer infections. |
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Shwachman-Diamond Sydrome
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Pancreatitis
Bone marrow stroma deficiency thus cannot help Neutrophil migrate or extravasate. INEFFECTIVE HEMATOPOEISIS. Must replace bone marrow. |
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Cyclic Granulocytopenia
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essentially 3 weeks of normal hematopoitic function and then 1 week of deficiency.
Need Bone marrow transplant. |
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C3 deficiency
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MOST SEVERE of all.
ALL PATHWAYS AFFECTED. |
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C2/4 deficiency
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NO classical or lectin pathway (Antibody mediated)
Only Alternative pathway is open. |
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Common Variable Immunodeficiency
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Most common in adults
GI disorders Generally ALL IMMUNOGLOBINS DECREASE. T cells do not proliferate. |
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autoimmune hemolytic anemia
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Type II
IgG 1/3 antibody Abenua/Hepatosplenomegaly Direct - AB on RBC Indirect Coomb's test - AB in serum Abnormal Spherocytes Increase in abnormal RBC count |
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Autoimmune thrombocytopenic Purpura
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Type II
Autoantibodies GPIIa/b Phagocytosis via IgG Decreased platelet counts and increased megakaryocytes. |
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Goodpasture Syndrome
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Type II
collagen type IV Anti-glomerular membrane Pulmonary and renal failure hypertension & edema C3 mediated opsonization of membrane |
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Rheumatic Fever
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Type II
IgG against M protein damage cardiac valves mimicry of strep |
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Pemphigus Vulgaris
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Type II
Peptide against desmoglein 1 / 3 Disrupt intraceullar adhesions Large loss of fluids and electrolytes. |
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Myasthenia Gravis
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Type II
Postsynaptic ACHr on endplates muscle weakness, eyes, thymus etc. rapid stimulation will cause muscle weakness 70% have enlarged lymph nodes with GERMINATION CENTErS. |
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Graves Disease
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Type II
Hyperthyroidism Mimicry of TSH receptor with agonists. High T3/4 |
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Systemic Lupus Erythematosus
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Type III
Epitope spreading Deposition of Anibody/antigen complexes in blood vessels Anti Double stranded DNA Ab complexes taken to TL 7/9 receptors and increase in IFN alpha. |
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Wegener's Granulomatosis
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Type III
Proteinase 3 cytoplasmic antibodies (cANCA) Fleeting lung shadow Cartilage disformities |
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Hashimoto's
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Type IV
CD8 cells antibody production to TPO/thyroglobulin small hard goiter |
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Multiple Sclerosis
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Type IV
Cytotoxic T cells killing oligodendrocytes via toxic (NO) substances. antibodies to MBP in CSF look for Plaque presence |
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Insulin Dependend Diabetes Mellitus
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Type IV
GAD 65 coxsackievirus mimicry and CTL of islet cells increased glucose levels, polyuria, polydipsia etc. Ser/Thr a negative charge in position 9 or AA 57 causes disease. |
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Spondyloarthropathies
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Type IV
HLA B27 CTL mediated causing inflammation of joints. |
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Rheumatoid Arthritis
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Mixed
Rheumatoid factor excessive lymphocyte penetration of joints, Pannus. destroying cartilage and bone. IgM produced against IgG Susceptibility in HLA around 67-70 AA position |
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Scleroderma Progressive
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Mixed
Cd4 T cells release IL 2,3,TNF and activate inflammatory cells Anti Scl 70 Anti Topoisomerase I stimulate proteins Organ |
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Scleroderma Crest
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anti Scl 70
anti-centromere CREST Detection of IgM Rheumatoid factors. microstomia, anti -topoisomerase I. CALCINOSIS Reynauds Esophageal Sclerodactely Telengiactiasis (winding of arteries) |
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Sjorgen Syndromes
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Antibodies against Ro and La
decrease in tears and saliva non erosive arthritis enlarged parotid gland Lip Biopsy |
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Dermatomyositis
Polymyositis |
attacking capillaries and muscle via Cd4 and MHC I presentation
muscle weakness muscle biopsy Elevated Creatine Kinase |
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Mixed Connective tissue disease
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anti-RNP/ U1-RNP
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