Path 1.3

Front 1

B-cell (Ab) deficiency = ? infection

Back 1

bacterial

Front 2

T cell deficiency = ? infections

Back 2

fungal
protozoal
viral

NO BACTERIA!!!!

Front 3

Phagocytic cell deficiency = ? infection

Back 3

systemic opportunisitic infections
skin infections
pyogenic infections (bacterial)

Front 4

Complement deficiency = ? infection

Back 4

PYOgenic infections (bacterial)

Front 5

9 month male has no mature B-cells in blood or plasmacytes in lymphoid tissue. What are the Ig levels?

What is the major diagnostic finding?
What disease is this?

Back 5

all 5 isotype Ab are extremely decreased

small / absent lymph nodes

X-linked infantile agammaglobulinemia (XLA, Bruton's disease)

Front 6

Pt has B-cells but low IgG levels (other Ab levels are normal)

What is the disease?
What is the cause?
Treatment?

Back 6

Transient Hypogammaglobulinemia

lack of help from CD4+ T helper cell

persists for 36 months and resolves spontaneously

Front 7

onset 13-35 yrs
B-cells present but NO PLASMACYTES
frequent bacterial, viral, and parasitic infections

Disease?
risk of malignancy?
relatives of pts have ?

Back 7

Common Variable Hypogammaglobulinemia (CVID)

autoimmune disease
malignancy - Gastric Cancer and Lymphoma

isolated IgA deficiency

Front 8

Most common Ab deficiency?

Back 8

IgA deficiency

Front 9

Pt has recurrent sinopulmonary infections and chronic diarrhea

allergies and autoimmune disease

Back 9

IgA deficiency

BEWARE OF BLOOD TRANFUSION WITH IgA CAN CAUSE ANAPHYLACTIC SHOCK

Front 10

What is the mechanism for Immunodeficiency with High IgM?

Back 10

B cells cannot switch from IgM to other isotypes


CD40L T cell defect

or

CD40 B cell defect

Front 11

Cranofacial anomalies
no Thymus = T-cell deficiency
w/ tetany and seizures
frequent infections

Back 11

congenital thymic aplasia (digeorge syndrome)

Front 12

chronic Candidasis infection with Endocrinopathy

what is the cause?

Back 12

cannot make Type I cells (IL-2/INF-gamma) to fight Candida

makes only Type 2 T-cells (IL-4/IL6) which are ineffective against fungi

Front 13

Defective lymphoid tissues - NO T or B cells

What is the disease?

mechanism?

Back 13

Sever Combined Immunodeficiency Disease (SCID)

X-Linked SCID - gamma chain of IL-2 receptor defect --lymphoid stem cells cannot mature

Adenosine Deaminase (ADA) enzyme deficiency -- ATP and dATP accumulate to fatal levels in lymphoid stem cells

Front 14

low IgM levels
High IgA

T-cell count normal but decreasing

abnormal platelets / thrombocytopenia and eczema

Back 14

Wiskott-Aldrich Syndrome (WAS)

Front 15

defective repair of DNA
-TCR genes mutate
-Heavy chain AB genes mutate

Disease?
what Ab is most likely low?
Susceptible to ?

Back 15

Ataxia-Telangiectasia

IgA is low (other isotypes are a little higher)

radiation damage
high incidence of lymphoma/leukemia

Front 16

Complement deficiency of ? ? ? leads to autoimmune disease?

Back 16

C1

C2

C4

Front 17

complement deficiency has increase autoimmune and bacterial infections (Gm-)?

Back 17

C3

Front 18

complement deficiency has increase infections with Neisseria meningitides (Gm+) and Gm- bacteria?

Back 18

C5, C6, C7, C8, C9

Front 19

Pt. has increased vascular permeability
swelling / edema of skin and mucus membranes

disease?
defect?
What is elevated?

Back 19

Hereditary Angioedema

C1-inhibitor (C1 esterase inhibitor) deficiency

C1qrs activation
Factor XII and Kallikrein (coagulation cascade)

Front 20

HIV infects ? cells

Back 20

CD4+ T cells

Front 21

Cytomegalovirus infects ? cells

Epstein Barr Virus infects ? cells

Back 21

T-cells

B-cells