Immunodeficiency Flashcards
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
