Immuno/ Allergy / Rheum

Question 1

B cells

Answer 1

target extracellular pathogens / encapsulated bacteria

Question 2

features of 1 b cell deficiency

Answer 2

H. influenz / strep pneumo
failure to respond to antibiotics
freq. URI after 6 mo (otitis media, sinusitis, pneumonia)

Question 3

X-linked agammaglobulinemia

Answer 3

after 7 mo of age
lost maternal antibodies
no B cells = severe life threatening GI infxns / encapsulated organisms

Question 4

common variable immunodeficiency

Answer 4

inherited disorder of hypogammaglobulinemia (igG/IgA)

• incr. incidence of lymphoma / autoimmune disease
• defective antibody formation

Question 5

selective IgA defic

Answer 5

can handle viral infxn

bacterial infxns of the resp / GI / urinary tract

Question 6

disorders of cell-mediated immunity

Answer 6

• inability of T cells to direct B antibody synthesis to T-cell specific antigens
• freq. recurrent infxn w/ opportunistic / low-grade organisms / viruses
• incr. incidence of autoimmune disorders / malignancies
• anergy

Question 7

lab tests in disorders of cell-mediated immunity

Answer 7

absolute lymphocyte count

• abnL mitogen stimulation response
• delayed hypersensitivity testing

Question 8

phagocytic disorders problem + lab tests

Answer 8

not enough neutrophils

-ANC, blood smear for blasts, antineutrophil antibodies

Question 9

phagocytic disorders presentation

Answer 9

cellulitis, skin abscess, furunculosis
stomatitis, gingivitis, rectal inflammation
PNA, sepsis

Question 10

phagocytic disorders / CGD problem

Answer 10

inability to kill intracellular bacteria 2/2 to failure to generate O2 metabolites (superoxide anion)

Question 11

chronic granulomatous disease tests

Answer 11

nitroblue tetrazolium test

DHR conversion test

Question 12

CGD sequelae

Answer 12

incr. susceptibility to infxns w/ catalase positive bacteria / fungi
chronic lymphadenitis, abscesses, granulomas, osteomyelitis

Question 13

complement disorders problem + test

Answer 13

impaired opsonization

• total hemolytic complement
• assays of classical/al. pathways

Question 14

complement disorders presentation

Answer 14

• recurrent bacterial infxns w/ encapsulated, extracellular organisms
• incr. susceptibility tomeningococcal / gonnococcal disease

Question 15

transient hypogammaglobulinemia of infancy

Answer 15

maternally derived immunoglobulins dec & are replaced by the child’s growing production - susceptible to infxn ages 6 - 12 wks

Question 16

signs of JIA

Answer 16

extra-articular signs (intermittent high fevers once/twice daily)

• toxic + profound malaise
• evanescent, nonpruritic salmon-colored rash
• hepatosplenomegaly, lymphadenopathy, signs of serositis / pericarditis
• labs: leukocytosis, thrombocytosis, anemia, elevated ESR/CRP
• very elevated ferritin
• no ANA/Rh factor