Immunology Flashcards
Recurrent infections with pyogenic bacteria indicate what kind of deficiency?
B-cell deficiency
State in which the immune system’s ability to fight infectious disease is compromised
Immunodeficiency
Recurrent infections with fungi, viruses or protozoa indicate what kind of deficiency?
T-cell deficiency
It is characterized by genetic susceptibility to infections since childhood?
Primary immunodeficiency (Ex: IgA deficiency, Bruton’s XLA)
It is characterized by acquired susceptibility to infection as a result of external processes or diseases
Secondary immunodeficiency (Ex: Malnutrition, Aging, Drugs, AIDS)
Very low levels of ALL immunoglobulins There is virtual absence of B cells due to tyrosine kinase mutation Cell-mediated immunity is normal
X-linked agammaglobulinemia or Bruton’s Agammaglobulinemia
Presents with: Recurrent pyogenic bacterial infections Recurrent enteroviral infections Recurrent giardiasis Affects male infants at about 6 months of age
X-linked agammaglobulinemia or Bruton’s agammaglobulinemia
What is the source of the problem with Bruton’s agammaglobulinemia? What is the treatment?
Tyrosine kinase mutation Pooled gamma globulin
There is failure of isotype switching There is recurrent bacterial sinus and lung infections
Selective IgA deficiency
Should you give gamma globulin to patients with Selective IgA deficiency? Why or why not?
No -form antibodies against foreign IgA -cross reaction depletes their already low IgA or may cause anaphylaxis
Defect in B-cell maturation to plasma cells Recurrent pyogenic bacterial infections Most common form of severe antibody deficiency affecting both children and adults Usually a diagnosis of exclusion What is this disease and what is the treatment?
Common Variable Immunodeficiency Pooled gamma globulin
What are the problems with DiGeorge Syndrome?
CATCH-22 Cardiac defect (TOF) Abnormal facies Thymic aplasia Cleft palate Hypocalcemia 22q11.2 chromosomal deletion
There is a profound deficit of T cells due to the failure of the development of thymus and parathyroids Humoral immunity is normal
DiGeorge Syndrome
What caused the thymus development failure in DiGeorge Syndrome?
Defect in the 3rd and 4th pharyngeal pouches
There is tetany due to hypocalcemia and prone to severe viral, fungal or protozoal infections during infancy What can explain this and what is the treatment?
DiGeorge Syndrome Transplant of fetal thymus (Note: this must come from a baby who is less than 14 weeks old)
What is the immunologic problem with Chronic Mucocutaneous Candidiasis and its treatment?
Specific T-cell deficiency for Candida albicans Tx:Azole antifungals (Note: other T and B cell functions are normal and it presents with recurrent candidiasis in the skin or mucous membranes of children)
What are the two mechanisms that can explain Severe Combined Immunodeficiency (SCID)?
1) X-linked: defect in IL-2 receptors in T-cells 2) Autosomal: Adenosine deaminase (ADA) deficiency
In SCID, there is a defect in IL-2receptors in T-cells. What is the consequence of this?
Major Histocompatibility Complex (MHC) II can’t be formed.
Presents with recurrent bacterial, viral, fungal and protozoal infection in early infancy (3mos). What is this and its treatment?
Severe combined immunodeficiency (SCID) Enclosure in a plastic bubble and bone marrow transplant.
Presents with recurrent pyogenic infections, eczema, and bleeding due to a low platelet count. It is x-linked and there is an inability to mount an IgM response What is the disease, what is the x-linked problem and what is the treatment?
-Wiskott-Aldrich Syndrome -Mutation in the WASP gene for actin filament assembly -Bone marrow transplant How do you TIE a WASP? Thrombocytopenia Infections Eczema WASP mutation
Autosomal recessive disease Mutations in DNA repair enzymes IgA deficiency Recurrent infections by 2 years of age What is the disease and treatment?
Ataxia-Telangiectasia Tx: supportive treatment (Note: also presents with Ataxia and Telangiectasia, pero give away kung ilalagay sa question) Mnemonic: ATI-atihan Ataxia Telangiectasia IgA deficiency
Autosomal recessive disease Failure of phagolysosomal fusion Faulty _____ which impairs neutrophil chemotaxis Recurrent pyogenic infections caused by staph or strep What is the disease, the blank and treatment?
Chediak-Higashi Syndrome Microtubules Antibiotics
There is a lack of NADPH oxidase activity but there is normal B and T cell activity Recurrent infections with catalase positive bacteria and fungi Widespread granulomas of unknown etiology What is the disease, diagnostic and treatment? Also, what catalase positive bacteria and specific fungi causes recurrent infections in these patients?
Disease: Chronic Granulomatous Disease Diagnostic: Nitroblue Tetrazolium Test Treatment: Antibiotics Catalase +: Staphylococcus spp. Fungi: A. fumigatus
Autosomal recessive disease Mutation in integrins Defective adhesion proteins on thensurface of phagocytes There is severe pyogenic infection in infancy and delayed separation of umbilical cord What is the disease, the adhesion protein and treatment?
Leukocyte Adhesion Deficiency (LAD) or Lazy Leukocyte Disease LFA-1 Antibiotics and bone marrow transplant
