Primary and Secondary Immunodeficiencies Flashcards
General clinical signs of immunodeficiencies
-infection; more severe persists longer, frequent recurrences, not responsive to rx
- physical/developmental disorders
- failure to thrive
- does achieve/maintain expected growth
How many respiratory infections does a “healthy” child have each year?
4-8 respiratory infections per year (10-12 years of age)
Average duration of infection =8 days»»can have symptoms for nearly one-half year of year.
What type of vaccinations should not be given to an immunodeficient child?
live virus vaccines
Primary care of child w/ immunodeficiency
- prompt and aggressive treatmenet of infection
- may need prophylactic rx
- if need blood transfusion use irradiated, leukocyte poor, virus free products
Laboratory test for Immunodeficiency?
CBC w/ peripheral smear
lymphocyte immunophenotyping
quantitative immunoglobulin
aby titers
protein electrophoresis
X-Linked Hypogammaglobulinemia (Burton’s)
- Primary/secondary?
- Type of deficiency
- cause
- SE
- symptoms
- when does this happen in life?
- treatment
- Primary/secondary- primary
- Type of deficiency - B cell
- cause- *aby deficiency, gene mutation encoding *Bruton tyrosine kinase (Btk)
- SE: *low levels of all Igs, absence of b-cells, normal T cell response
- Symptoms- recurrent *pyogenic bacterial infections, failure to thrive, absence of tonsil and cervical lymph nodes in recurrent otitis?
- when does this happen in life? young boys 6mo of age, female carriers are immunocompetent
- treatment- replacement of IgG through IVIG, SCIG (sub cutaneous immunoglobulin)
Selective IgA Immunoglobulin Deficiency
- Primary/secondary?
- Type of deficiency
- cause
- SE
- symptoms
- when does this happen in life?
- treatment
- Primary/secondary? Primary
- Type of deficiency- B cell deficient
- cause- inability of b cells to differentiate and mature into *IgA (low levels) secreting plasma cells
- SE- recurrent *sinopulmonary infections, GI, conjunctiva, and respiratory tract infections
- symptoms- 90% are asymptomatic
- when does this happen in life? diagnosis only happens after age 4. Most common in adults,
- treatment- discontinue med causing decreased IgA, none, abx therapy for recurrent infections, should not be treated w/ gamma globulin(anaphylactic hypersensitivity may occur; cross reaction will deplete their already low levels of IgA)
Common Variable Immunodeficiency (CVID)
- Primary/secondary?
- Type of deficiency
- cause
- SE
- symptoms
- when does this happen in life?
- treatment
- Primary/secondary?- primary
- Type of deficiency- b cell
- cause- b cells w/ impaired ability to mature cause unknown.
- SE- defective immunoglobulin production, *low serum IgG, IgA, IgM
- symptoms; recurrent *sinopulmonary infections (bronchitis, otitis, TB, fungal)
- when does this happen in life? 2nd and 4th decade in life, 5-7 years between onset of symptoms and diagnosis
- treatment- IVIG or SQIG, abx
Important T cell Functions
control viral disease
control fungal disease
provide helper function to b cells for effective humoral response
Thymic Aplasia (DiGeorges Syndrome)
- Primary/secondary?
- Type of deficiency
- cause
- SE
- symptoms
- when does this happen in life?
- treatment
- Primary/secondary? Primary
- Type of deficiency- T cell
- cause:heterozygous chromosomal deletion at 22 leading to absence of T cells
-SE; absence of development of thymus, thyroid glands, parathyroid glands
(parts of pharyngeal pouch)
hypoparathyroidism»tetany
- symptoms; cardiac abnormalities, facial abnormalities (low set ears, down-slanting eyes, short philtrum)
- when does this happen in life?- infants early in life
- treatment: immediate tx of cardiac abnormality, thymus transplant, control metabolic abnormalities
Severe Combined Immunodeficiency (SCID)
- Primary/secondary?
- Type of deficiency
- cause
- SE
- symptoms
- when does this happen in life?
- treatment
- Primary/secondary?- primary
- Type of deficiency- *B & Tcells (may be defective, low in number, or completely absent)
- cause; group of inherited diseases caused by mutations in different genes crucial in the development of B & T cells, may be x-linked (75%) or autosomal
- SE; absence of normal thymic tissue and other tissues devoid of lymphocytes, Ig levels low, tonsil & lymph nodes absent
- symptoms; failure to thrive, viral and bacterial, fungal, protozoal infections
- when does this happen in life? within 1st year of life, resulting in death usually.
- treatment- DO NOT GIVE LIVE VIRAL VACCINE, isolation in a plastic bubble (main intervention), bone marrrow or cord blood transplant, IVIG, enzyme replacement therapy
Wiskott-Aldrich
Ataxia-Telangiectasia
Hereditary Angioedema
- Primary/secondary?
- Type of deficiency
- cause
- SE
- symptoms
- when does this happen in life?
- treatment
- Primary/secondary? Primary
- Type of deficiency- Complement associated deficiency
- cause; lack of c’1 protease inhibitor resulting in too much C1, excess C3a leading to inflamm process»>edma
- SE; often need to be intubated
- symptoms; edema, especially laryngeal
- when does this happen in life?
- treatment; immediate airway assessment (intubation), Human plasma C1 inhibitor, FFP
Malnutrition
- Primary/secondary?
- cause
- SE
- Primary/secondary?- secondary
- cause: PEM-Kwashiorkor or Marasmus, leading to decreased Vit. A, C, B12, B6
- SE; decreased T cells, increased NK, normal/increased Igs, decreased Ig response, decreased phagocytic function
- most common immunodeficiency world wide!
Infections
- Primary/secondary?
- cause
- SE
-Primary/secondary? secondary
- cause: viral, bacterial
ex. HIV, Measles, Parasitic Infections
-SE:
HIV- decreased CD4+ T cells,
Measles- diminished Aby production
Parasite: alteration of Mf functions (inhibit chemotaxis and phagocytosis) induction of Tsuppressor cells
Cancer -Primary/secondary? -cause -immunologic abnormalities associated w/ cancer -
-Primary/secondary?- Secondary
- cause: healthy cells stop functioning and maturing properly
- Immune deficiency may be secondary to chemo, radiation, or cancer
-abnormalities- multiple myeloma: CD4+ t cells decrease, deficits in complement and neutrophil function
