5. Defensive Breakdown Flashcards
What type of inheritance does B cell congenital immunodeficiency have?
X linked recessive.
What is the presentation of B cell congenital immunodeficiency?
Early presentation, no tonsils. Get recurrent infections.
How are immunoglobulin levels impacted by B cell congenital immunodeficiency?
Low IgA and IgM.
What is B cell insufficiency?
Failure of B cells to mature.
What are the infections patients with B cell insufficiency get?
Recurrent bacterial, usually respiratory tract infections.
How is B cell insufficiency treated?
Ig transplantation.
What immunodeficiency is seen in DiGeorge syndrome?
T cell.
How does DiGeorge syndrome cause immunodeficiency?
Absent thymus and other congenital defects so T cell deficiency.
Which infections are T cell deficient patients prone to?
Fungal and viral infections primarily.
What is severe combined immunodeficiency?
Recessive condition causing low B and T cells.
How does SCID present?
Chronic thrush, extensive diaper rush, failure to thrive, recurrent fungal, bacterial and viral infections.
How is SCID managed?
Bone marrow transplant.
What is ataxia telangiectasia?
Recessive immunodeficiency with thymic hypoplasia and low B cells which is fatal by aged 20.
How is ataxia telangiectasia treated?
With bone marrow transplantation.
What is chronic granulomatous disease?
X linked recessive disorder leading to persistent infections of the skin, respiratory and GI tract due to defective bacterial killing by neutrophils.
What are the acquired B cell immunodeficiencies?
Hypogammaglobulinaemia, chronic lymphatic leukaemia, lymphoproliferative disorders, myeloma, nephrotic syndrome.
What are the acquired T cell immunodeficiencies?
HIV, chemotherapy, Hodgkin’s disease, immunosuppression.
What are the acquired B and T cell immunodeficiencies?
Radiotherapy, chronic lymphatic leukaemia, malnutrition.
What are the acquired neutrophil immunodeficiencies?
Neutropenia (not enough), myelodysplasia (not functioning).
What does the HIV virus infect?
T helper cells via CD4 molecules, this affects T and B cell responses.
What is the result of the immunodeficiency of HIV?
Opportunistic infections.
What are the fungal infections seen in HIV?
Pneumocystis jiroveci (pneumonia), candida albicans (thrush), aspergillus fumigatus (pneumonia), histoplasma capsulatum (disseminated), cryptococcus neoformans (meningoencephalitis, pneumonia).
What are the mycobacterial infections in AIDS?
TB, MAI (mycobacterium avium-intracellular).
What are the parasitic infections seen in AIDS?
Cryptosporidia (GIT), isospora (colon), toxoplasma gondii (CNS, eyes, lymph nodes).
