Immunodeficiency Flashcards
(21 cards)
B cell (antibody) dysfunction
Recurrent, pyogenic infections
T cell function
Chronic/recurrent candida infections
Low lymphocyte count
Wiskott Aldrich syndrome
X linked recessive Eczema Thrombocytopenia Immunodeficiency - OM, pneumonia Hematologic malignancies
Severe Combined Immunodeficiency
Absence of lymphoid tissue
Dysfunctional B, T cell function
T, B, NK cell subset analysis
First 3 mos - diarrhea, dermatitis, FTT
Life threatening infections
DiGeorge syndrome
Absence of thymic shadow
Hypocalcemia
May present like SCID
Ataxia Telangiectasia
Ataxia
Discoloration of the conjunctiva
Frequent sinus infections
Bruton’s disease
X-linked agammaglobinemia
B cells
T cells elevated
Immunoglobulin all low
Absence of lymphoid tissue
Encapsulated bacteria
Common Variable Immunodeficiency
Defective antibody response B and T cells Recurrent upper/lower respiratory infections Recurrent HSV, zoster infections Increased risk of lymphoma
Complement deficiency
Autosomal recessive
Meningococcal infection
X Linked Hyper IgM syndrome
High IgM; low IgG, IgA, IgE
Frequent otitis, sinopulmonary infections
PCP in absence of HIV
Job syndrome
Hyper IgE syndrome
Eosinophilia
Eczema
Elevated IgE
S aureus indections
Sinopulmonary infections
Transient Hypogammaglobinemia of Infancy
Outgrow by 3-6 mos
Low IgG
Chronic Granulomatous Disease
X linked, autosomal recessive
Abnormal phagocytic function - resp burst
Skin, GI, lungs, liver, LN, spleen
Catalase positive
S aureus, Serratia, Burkholderia, Nocardia, Aspergillus
Nitroblue tetrazolium test
Leukocyte Adhesion Deficiency
Defect in chemotaxis
Elevated WBCs Delayed wound healing Delayed umbilical cord separation No pus, minimal inflammation Omphalitis, indolent skin infections
HIV
Decreased CD4
Nitroblue tetrazolium
Neutrophil activity
Normal = blue, abnormal = colorless
Chronic granulomatous disease
CH50
Complement system
Repeated serious bacterial infections
TB, Candida skin test
Cell mediated immunity, T cell defects
AIDS
Immunoglobulin levels
Humoral system
Less serious infections
Rarely before 6 mos (maternal immunity)
Specific antibody tests
IgG normal
Humoral defect - subclasses from vaccines
Rebuck skin window
Cell migration in LAD
