I2: Immune Deficiencies

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defects in antibodies, complement, and phagocytic cells leads to

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extracellular bacterial infections after 6 months

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T cell defects cause

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infections with intracellular pathogens (viral, fungal, bacterial)- early symptoms

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antibody decifiency states show

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recurrent pyogenic bacterial infections

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antibody deficiencies are in association with

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autoimmunity and secondary to malignancy of lymphocytes

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B cell deficiences are diganosed strongly by

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isohemagglutinin titer
antibody response to immunization

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Other B cell deficiencies detection methods

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serum protein electrophoresis
quantitative immunoglobulins

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treatment of B cell deficiencies

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antibiotics as needed
IVIG
stem cell reconstitution

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How is IVIG contraindictated in selective IgA deficiency

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Patients deficient in IgA have anti-IgA antibodies and so with IVIG therapy anaphylactoid reactions may occur (type III)

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selective IgA deficiency occurs when there is

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a block in plasma cell differentiation or secretion of IgA

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why are half the IgA patients asymptomatic?

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The Ig receptor can bind IgA ad igM so in half the cases, IgM can compensate for missing secretpry IgA

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transient hypogammaglobunemia

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onset at 6-9 months with normal numbers of B and T cells
delayed onset of Ig production

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signs of transient hypogammaglobinemia

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otitis media, pneumonias, diarrhea

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X-linked agammaglobulinemia is a ____ disease present in ____

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congenital
males

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signs of x-linked agammaglobulinemia

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otitis media, diarrhea, pneumonia, sinusitis

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X-linked agammaglobulinemia results from

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impaired bruton's tyrosine kinase expression in pre-B cells

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in X-linked agammaglobulinemia there are

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reduced-to-absent B cells in peripheral lymphoid tissues

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DiGeorge syndrome is a

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rare congenital thymic hypoplasia

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symptoms of DiGeorge syndrome

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lymphopenia, moderate hypogammaglobulinemia, Ca++ imbalance, cardiac disease

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DiGeorge occurs at

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early age with viral and fungal infections

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treatment of DiGeorge syndrome

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thymus transplantation

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methods to diagnose T cell deficiencies

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CBC
lymphocyte counts
CD3,4,8
CD4/CD8 ratios
HIV
intraderma skin test
invitro hyperproliferation tests
MLR

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bare lymphocyte syndrome 1 is caused by a

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CIITA mutation (no MHC II) so there is poor CD4+ activation and development

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bare lymphocyte syndrome 2 is caused by a

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TAP deficiency . there is no MHC I which leads to poor CD8+ development

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severe combined immune deficienct is due to an ____ deficienct

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IL-2gR, JAK, adenosine deaminase, RAG-1/2

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treatment of SCID

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stem cell transplant

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Adenosine deaminase deiciency leads to

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T, B, NK cell toxicity

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treatment of ADA

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gene therapy

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gamma c chain deficiency leads to

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defective lymphocyte development and poor B cell isotype switching

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leukocyte adhesion defect leads to

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altered neutrophil extravasation/opsonophagocytosis

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Leukocyte adhesion defects treatment

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antibiotics/stem cell

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chronic granulomatous disease is caused by

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a decreased NADPH oxidase proteins

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CGD symptoms

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fungal/bacterial infections with granulomas

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treatment of CGD

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neutrophil transfusions, recombinant IFNgamma- stem cells

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cyclic neutropenia caused by

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deficiency in neutrophil elastase

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cyclic neutropenia is treated with

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recombinant CSF-G therapy