Humoral Immune Deficiencies Flashcards
What is the most common primary immunodeficiency?
IgA Deficiency
Over 80% are asymptomatic
NOT and indication for IVIG replacement
What is Bruton’s X-linked Agammaglobulinemia (XLA)?
Primary affects B cells. T cells are ELEVATED
IgM, IgG, IgA, and IgE are LOW
Tonsils, Adenoids, Peyer’s Patches, Spleen, and Peripheral lymph nodes are SMALL
Get infections with encapsulated pyogenic bacteria: Strep pneumo, H. flu
At risk for bronchiectasis and chronic pulmonary insufficiency
How do you diagnosis and treat Bruton’s X-linked Agammaglobulinemia (XLA)?
Diagnosis-
Measure immunoglobulin levels. When low then confirm by measuring B and T cells
Treatment-
Need IVIG
DO NOT GIVE LIVE VIRAL VACCINES
What is Common Variable Immunodeficiency (CVD)?
Most common CLINICALLY SIGNIFICANT antibody deficiency.
B cells cannot differentiate into plasma cells, all Igs are deficient. T cell defects present too
How do pts with Common Variable Immunodeficiency (CVD) present?
NORMAL CBCs
Recurrent infections of the upper and lower resp tract
Frequent GI sxs (chronic diarrhea and malabsorption)
Recurrent herpes and zoster infections
Increased risk for LYMPHOMA (EBV associated).
Associated with autoimmune disease (RA, cytopenias, thyroid abnormalities)
How do you treat pts with Common Variable Immunodeficiency (CVD) present?
Treatment-
IVIG
DO NOT GIVE LIVE VIRAL VACCINES
What is X-linked Hyper IgM Syndrome? How does it present?
Disruption of B cell differentiation due to T CELL abnormality. Can’t convert IgM to IgG.
Male 6-12 mo with frequent otitis and sinopulmonary infections, as well as diarrhea
HALLMARK: lymphoid hypertrophy despite antibody deficiency
Presence of Pneumocystis carinii without HIV infection.
How do you treat patients with X-linked Hyper IgM Syndrome?
Treatment-
IVIG
DO NOT GIVE LIVE VIRAL VACCINES
What is Job Syndrome/Hyper IgE?
Eosinophilia Eczema Elevated IgE *recurrent skin infections *chronic thrush *abnormal facies *multiple fractures/skeletal abnormalities
Can be mistaken for atopic dermatitis, but you won’t have skeletal abn or weird facies
Different from Wiskott-Aldrich because you’ll have a milder rash and they have bleeding due to thrombocytopenia
S. aureus is the main bug!
How do you treat Job Syndrome/Hyper IgE?
Treatment: antibiotics and steroids
What is transient hypogammaglobulinemia of infancy?
Decreased T-helper function that leads to lower than severely LOW IgG and +/- low IgA. IgM NORMAL.
Presents by 6 mo and kids outgrow by 3-6 yo
