Term
| A T-Cell defect causes what to result from bacterial infection? |
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Definition
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Term
| A B-Cell defect causes what bacterial infections to predominate? |
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Definition
| Strep, Staph, Heamophilus |
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Term
| A Granulocyte defect causes what infections to predominate? |
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Definition
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Term
| A Compliment defect causes what infections to predominate? |
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Definition
| Neisseria, other pyogenics |
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Term
| A T-Cell defect can lead to what viral infections? |
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Definition
| CMV, EBV, severe varicella, chronic respiratory and intestinal viral infection |
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Term
| A B-Cell defect can lead to what viral infection? |
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Definition
| Enteroviral encephalitis, echovirus, poliovirus, coxackivirus (all neutralized by antibodies) |
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Term
| A T-Cell defect can lead to what fungal and parasitic infections? |
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Definition
| Candida, Pneumocystis jiroveci |
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Term
| A B-Cell Defect can lead to what fungal or parasitic complications? |
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Definition
| Severe Intestinal Giardiasis |
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Term
| A Granulocyte defect can lead to what fungal and parasitic infections? |
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Definition
| Candida, Nocardia, Aspergillus |
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Term
| What are the special presenting features of T-Cell defects? |
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Definition
| Aggressive disease, opportunistic pathogens, failure to clear infection fully |
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Term
| A B-Cell defect has what common presenting features? |
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Definition
| Recurrent sinopulmonary infections, sepsis, chronic meningitis |
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Term
| Where is the defect located in X-Linked Agammaglobulinemia |
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Definition
| Long arm of X Chromosome Xq21.22 encoding for Bruton Tyrosine Kinase needed to transduce signals from the Ig complex in the Pre-B Phase |
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Term
| What are some common presentations of X-Linked Agammaglobulinemia? |
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Definition
| After 6 mo...recurrent bacterial infection of respiratory tract, otitis media, sinusitis, pharyngitis, bronchitis |
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Term
| What is a common site of initial viral infection in X-linked agammaglobulinemia |
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Definition
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Term
| Describe the difference in B cell numbers between Common Variable Immunodeficiency and X-Linked Agammglobulinemia? |
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Definition
| B Cells are Absent in X-linked and present in normal numbers in CVI |
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Term
| Describe the difference in appearance between germinal centers in X-linked agammaglobulinemia and Common Variable Imunnodeficiency and the cause of this difference |
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Definition
| Germinal centers atrophic in X-Linked and hypertrophic in CVI dude to lack of Ig regulatory feedback |
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Term
| B-Cell defects are associated with higher incidence of what kind of disorder? |
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Definition
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Term
| Hyper IgM is characterized by failure on what process of B-Cell activation |
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Definition
| Binding of CD40L is deficient leading to lack of isotype switching and affinity maturation |
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Term
| What primary clinical findings are present in a patient with HyperIgM? |
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Definition
| Autoimmune Hemolytic Anemia, Throbocytopenia, neutropenia, Infiltration of plasma cells in GI tract, recurrent pyogenic infection, Pneumocystis Jiroveci. LABs=Normal/High IgM, Low IgG, No IgE or IgA |
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Term
| DiGeorge syndrome results from failure of which branchial arches to develop? |
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Definition
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Term
| What structures are potentially missing in DiGeorge syndrome? |
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Definition
| thymus, parathyroids, clear cells of thyroid and ultimobranchial body |
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Term
| What clinical presentations are associated with Digeorge syndrome? |
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Definition
| Tetany (PTH), variable loss of t-cell mediated immunity, congenital defects of heart and great vessels, abnormal facies |
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Term
| What is the genetic basis of Digeorge syndrome? Is it Autosomal, sex-linked? Dominant or Recessive? |
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Definition
| 22q11 deletion, not familial |
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Term
| What is the most common method of inheritance in SCID? |
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Definition
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Term
| What defect is the cause of X-linked SCID? |
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Definition
| mutation in gamma chain of cytokine receptors resulting in faulty signalling and impaired T cell development and B cell activation (lack of CD4+) |
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Term
| What is the most common cause of autosomal recessive SCID? |
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Definition
| Adenosine Deaminase Deficiency (ADA) leads to accumulations of toxic metabolites in rabidly dividing immature lymphocytes |
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Term
| In which major etiology of SCID would one find intact Hassal's corpuscles in the thymus? |
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Definition
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Term
| What treatments are available for SCID? |
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Definition
| Bone Marrow Transplant, Gene Therapy |
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Term
| What is the mechanism of Wiskott-Aldritch syndrome? |
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Definition
| mutation of X11.23 encoding for a protein that links membrane receptors to cytoskeletal elements |
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Term
| What are the clinical findings in Wiskott-Aldrich Syndrome? |
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Definition
| eczema, vulnerability to recurrent infection |
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Term
| What are the laboratory findings in Wiskott-Aldrich Syndrome? |
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Definition
| Progressive depletion of T-cells, thrombocytopenia, low IgM, normal IgG, High IgE and IgA |
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Term
| What is the treatment for Wiskott-Aldrich syndrome? |
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Definition
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Term
| What condition is Wiskott-Aldrich syndrome commonly associated with? |
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Definition
| non-Hodgkin B-cell lymphomas |
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Term
| What is the most common deficiency of the complement? |
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Definition
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Term
| What is the main manifestation of early classical complement (C1, C2, C4) deficiency? |
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Definition
| Increased risk of SLE, does not affect susceptibility to infection |
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Term
| Why is C3 deficiency the most sever of complement disorders? |
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Definition
| C3 has functions in both the alternative and classical pathways |
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Term
| What kidney pathology is associated with C3 deficiency? |
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Definition
| immune complex mediate glomerulonephritis |
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Term
| Late phase compliment protein deficiency causes defects in formation of _________? This leads to recurrent infection with _______ |
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Definition
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Term
| What is the cause of hereditary angioedema? |
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Definition
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Term
| What is the genetic pattern of C1 inhibitor deficiency? |
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Definition
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Term
| What are the targets of the C1 protease inhibitor? |
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Definition
| C1r and C1s, factor XII, the kallikrein system |
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Term
| What is the largest demographic of HIV infected individuals? |
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Definition
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Term
| What is the second largest group of people affected by AIDS? |
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Definition
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Term
| What are the 3 main modes of HIV spread? |
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Definition
| Sexual contact (75%), parenternal contact, mother-child contact |
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Term
| What are the three viral enzymes included inside the capsule of HIV? |
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Definition
| protease, reverse transcriptase, integrase |
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Term
| For what cells does HIV display particular tropism and why? |
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Definition
| Macrophages, Dendritic Cells, and CD4+ T-Cells due to tropism for CD4+ cell surface proteins |
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Term
| In addition to CD4+ what other compounds are required for HIV binding and injection? |
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Definition
| CCR5 (utilized by R5 strains) or CXCR4 (utilized by X4 strains) some strains are dual trophic |
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Term
| What ligand on the HIV virus particle first binds CD4+? |
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Definition
| gp120 envelope glycoprotein |
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Term
| Which HIV protein is directly responsible for penetration of and fusion with the host cell? |
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Definition
| gp41 (fution peptide at tip revealed by CCR5 or CXCR4 coreceptor binding on gp120) |
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Term
| HIV is unsuccessful in infecting which of these varieties of T-Cells? Naive, Activated, Memory |
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Definition
| Naive, (They have APOBEC3G which induces C-to-U mutations in viral DNA) |
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Term
| What intermediary triggers activation of latent HIV infection in CD4+ T-Cells? |
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Definition
| Cytokines activate nf-kB which induces replication of HIV genome |
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Term
| How are monocyte and macrophage functions altered in AIDS? |
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Definition
| Decreased chemotaxis and phagocytosis, decreased Class II HLA expression, Diminished capacity to present antigen to T cells |
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Term
| impaired B Cell funtion dminished humoral immunity in AIDS patients, leaving them particularly vulnerable to what kind of bacteria? |
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Definition
| Encapsulated....like H influenzae and S Pneumonia |
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Term
| What protozoal and helminthic infections are associated with HIV infection? |
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Definition
| Cryptosporidiosis, isosporidiosis(enteritis), Toxoplasmosis(pneumonia or CNS infection) |
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Term
| What fungal infections are associated with AIDS? |
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Definition
| Pneumocystosis (pneumonia, disseminated infection), Candidaisis, Cryptococcosis (CNS), Coccidioidomycosis, Histoplamosis |
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Term
| What bacterial infections are commonly associated with AIDS? |
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Definition
| Mycobaterosis avium-intracellulare or tuburculosis, Nocardiosis (pneumonia, meningitis, disseminated) Salmonella |
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Term
| What viral infections are associated with AIDS? |
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Definition
| CMV, Herpes Simplex, varicella-Zoster, Progressive multifocal leukoencephalopathy |
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Term
| What is the major manifestation of Cryptococcosis in AIDS patients? |
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Definition
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Term
| What is the primary causitive agent of CNS mass lesions in AIDS patients |
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Definition
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|
Term
| What is the most common neoplasm in AIDS patients? |
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Definition
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|
Term
| What is the direct cause of Kaposi Sarcoma? |
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Definition
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|
Term
| Which AIDS related lymphoma class is most common? Systemic, CNS or body cavity? |
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Definition
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