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18 Cards in this Set
- Front
- Back
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A 6 month old patient presents w/ recurrent bacterial infections.
Lab results show normal pro-B cells, decreased B-cell maturation, decreased number of B cells, and decreased immunoglobulins of all classes. What defect is present in this patient? |
Defect in BTK
(blocks B-cell differentiation/ maturation) "Bruton's agammaglobulinemia" |
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A patient presents with severe pyogenic infections early in life. Lab results show increased IgM, very low IgG, IgA, IgE.
What is the defect present in this patient? |
Defective CD40L on helper T cells = inability to class switch.
"Hyper-IgM syndrome" |
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A patient presents w/ recurrent viral/fungal infections, congenital heart defects, and great vessel defects.
The lab results show decreased T cells, decreased PTH, and decreased Ca2+. What is the defect present in this patient? |
This patient's thymus gland failed to develop-- Thymic aplasia
"DiGeorge syndrome" |
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A patient presents with disseminated mycobacterial infections.
Lab results show decreased IFN-y. What defect is most likely preset in this patient? |
IL-12 receptor deficiency, resulting in decreased Th1 response.
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A patient presents with Candida albicans infections of skin and mucous membranes.
Which type of cell is most likely dysfunctional? |
T-cell dysfunction
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A patient presents w/ recurrent viral, bacterial, fungal, and protozoal infections.
Lab results show decreased IL-2R and increased adenine. What defects are possible in this patient? What disease is most likely present? |
Possibilities:
1. Defective IL-2 receptor 2. Adenosine deaminase deficiency 3. Failure to synthesize MHC II antigens Major problem: very few T and B cells are present in the this patient. Decreased IL-2R => decreased T-cell activation. Increased adenine => toxic to B and T cells. Failure to synthesize MHC II antigens => CD4+ T cells can't mature. Disease: SCID (Severe Combined Immunodeficiency) |
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A patient presents w/ recurrent bacterial infections, absent pus formation, and delayed separation of umbilicus.
Lab reports show neutrophilia. What defect is most likely present? |
LFA-1 integrin (CD18) defect
"Leukocyte adhesion deficiency" |
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A patient presents w/ recurrent pyogenic infections by staphylococci and streptococci; partial albinism, and peripheral neuropathy.
What defect is most likely present? |
Lysosomal enzymes aren't being released-- inadequate phagocytosis.
"Chediak-Higashi syndrome" |
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A patient shows increased susceptibility to catalse-+ organisms (S. aureus, E. coli, Aspergillus, Klebsellia, and Serratia).
A negative nitroblue tetrazolium dye reduction test comes up in the lab results. What disease is most likely present? |
Chronic granulomatous disease.
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Recurrent Neisseria infections likely would indicate what sort of deficiency?
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Some sort of complement deficiency (especially C5 - C9)
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A patient presents with recurrent episodes of severe edema that seem to be influenced by stress. The swelling is never painful, itchy, or red, and usually disappears within several days.
Subsequent lab work shows decreased serum levels of C4 and C2. What would be an appropriate diagnosis? |
Hereditary Angioneurotic Edema
Deficiency in C1 INH (leads to over production of bradykinin, a potent vasodilator) Treat w/ plasma or C1 INH concentrate and/or androgen (Note: this could also be caused by a more rare mutation in factor XII of coagulation cascade leading to over production of bradykinin). |
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If a patient's lab results show hemolytic anemia and a defect in expression of MCP, DAF, and CD59, what is this patient suffering from?
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Paroxysmal nocturnal hemoglobinuria
Inability to form glycolipid linkages (which typically anchor DAF and MCP to cell). Complement activation is unchecked, so the MACs cannot be deactivated, which can lead to lysis of erythrocytes. (abnormally sensitive to complement-mediated lysis) |
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What would be an effective therapy for some inflammatory diseases?
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IVIG
(Intravenous Immunoglobulin) Works by engaging the inhibitory Fc receptor on B cells (and perhaps on dendritic cells), thereby suppressing pathologic immune responses |
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What would be a therapeutic solution for a child under 2 years old who does not respond well to polysaccharide antigens (T-independent antigens).
(This problem can result in Hib meningitis: Haemophilus influenzae type b meningitis) |
Solution:
Administer Hapten-carrier conjugates Make this vaccine by converting the PS capsule to a T-dependent antigen by conjugating it to proteins (diptheria or tetanus toxoids). |
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A patient who has undergone a splenectomy is susceptible to disseminated infections by what type of bacteria?
Why? |
More susceptible to encapsulated bacteria.
The spleen contains large numbers of phagocytes and is an important site of phagocytic clearance of opsonized bacteria. |
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Recurrent Niesseria infections are usually a sign of what type of deficiency?
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Complement deficiency (usually in MAC or complement in alternative pathway)
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A patient has suffered from recurrent infections, prolonged episodes of diarrhea, bacterial pneumonias, and several fungal infections.
Lab tests revealed: 1. No defects in total number of neutrophiles, monocytes, or B and T cells 2. Decreased CD4+ T cells 3. Low serum Ig levels. 4. Phagocytes indicated normal respiratory burst. What would be a logical diagnosis for this patient? |
Bare Lymphocyte Syndrome
MHC Class II deficiency (CD4+ T cells require expression of MHC Class II molecules on thymic epithelial cells in order to mature. CD4+ T cells are required for antibody production and macrophage activation) |
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A woman gives birth to an infant who is lethargic, jaundiced, and severely anemic and has an enlarged liver and spleen. The mother is RhD (-) and the father is homozygous RhD (+). This is their 2nd child.
What is the most logical diagnosis of this infant? What medication should have been used to prevent this? |
Hemolytic Disease of Newborn
(Erythroblastosis fetalis) During mother's first pregnancy, fetal red cells entered her circulation at birth. These RBCs were recognized by the mother's immune system, and antibodies were produced against Rh(+) RBCs. During her 2nd pregnancy, these antibodies crossed the placenta and attacked the fetus's RBCs. Use RhoGam (anti-RhD IgD Ab) |
