Notes in B/T Cells

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Published	10/08/2024	Immunodeficiencies are divided into {{c1::primary}} and {{c1::seconday}}.
Published	10/08/2024	2+ evnts of serious sinusitis or pneumonia in 1 year OR 2+ events of sepsis or meningitis in a lifetime should raise suspicion for an {{c1::immunodefi…
Published	10/08/2024	Opportunistic pathogens should raise suspicion for {{c1::immunodeficiency}}.
Published	10/08/2024	{{c1::HIV}}, {{c1::diabetes mellitus}}, {{c1::splenectomy}} and {{c1::certain medications}} can cause secondary immunodeficiencies.
Published	10/08/2024	Antibody disorders are the most common primary immunodeficiencies. The most common of these antibody disorders are:{{c1::Selective IgA deficiency}}{{c…
Published	10/08/2024	A selective IgA deficiency causes {{c1::decreased protection of the mucous membranes e.g. GI & Respiratory tracts}}
Published	10/08/2024	Symptoms of IgA deficiency include {{c1::Recurrent sinopulmonary or GI infections}}{{c1::Increased frequency of Atopy, Asthma, Autoimmune Dx}}{{c1::Se…
Published	10/08/2024	Diagnosis of IgA deficiency:{{c1::IgA <7mg/dL and normal IgG and IgM}}
Published	10/08/2024	Treatment of IgA deficiency:{{c1::Monitoring}}{{c1::No treatment available}}{{c1::Be careful with blood transfusions! Washed erythrocytes should be us…
Published	10/08/2024	X-linked agammaglobulinemia occurs due to {{c1::failure of B-cell maturation causing an absence of B cells and immunoglobulins!}}Its X-linked, therefo…
Published	10/08/2024	Symptoms of X-linked agammaglobulinemia:{{c1::Recurrent/severe bacterial infections: otitis media, bronchitis, pneumonia, pharyngitis, sinusitis}}More…
Published	10/08/2024	Diagnosis of X-linked agammaglobulinemia:{{c1::IgG level of <200mg/dL}}or{{c1::IgG + IgM + IgA of <400mg/dL}}or{{c1::Absence of IgM or IgA after…
Published	10/08/2024	Treatment for X-linked agammaglobulinemia{{c1::Lifelong monthly IVIg infusions ensuring passive immunity}}
Published	10/08/2024	Hyper-IgM syndrome is caused due to {{c1::a defect in the CD40 ligand therefore preventing B-cell class switching}} This is {{c1::x-linked}} thus…
Published	10/08/2024	Symptoms of Hyper-IgM syndrome:Predisposition to {{c1::fungal}}, {{c1::protozoal}}, and {{c1::viral}} infections, also {{c1::encapsulated bacteria}}Oc…
Published	10/08/2024	Diagnosis of Hyper-IgM syndrome:Increased {{c1::IgM}} and decreased {{c1::IgG, IgA & IgE}} levelsconfirmed with {{c1::flow cytometry: decreased CD…
Published	10/08/2024	Treatment of Hyper-IgM syndrome includes {{c1::lifelong IVIg infusions dependant on weight}}
Published	10/08/2024	DiGeorge syndrome (22q11.2 deletion) involves a {{c2::T-cell immunodeficiency}} that cause {{c2::developmental midline defects}}Developmental midline …
Published	10/08/2024	There is a T-cell deficiency in DiGeorge syndrome due to {{c1::thymic hypoplasia}}
Published	10/08/2024	Symptoms of DiGeorge syndrome regarding immunodeficiency include {{c1::recurrent infections of VZV, Candida, PJP, and Mycobacteria}}
Published	10/08/2024	Diagnosis of DiGeorge immunodeficiency:{{c1::Lymphocyte count of <1500 cells/yL in 5+ years}}{{c1::Function of T-cells test e.g. delayed hypersensi…
Published	10/08/2024	Severe Combined ImmunoDeficiency disorder is the most severe type of immunodeficiency as it lacks both {{c1::T-cells AND antibodies}}.
Published	10/08/2024	Symptoms of SCID include (3) {{c1::chronic diarreha, failure to thrive, and extreme suceptibility to infections, specifically encapsulated bacteria, e…
Published	10/08/2024	Diagnosis of SCID:{{c1::Absolute lymphocyte cound of <2500 cells/mm3}}{{c1::Flow cytometry: T-cells are less than 20% of cells}}{{c1::Response to m…
Published	10/08/2024	Treatment of SCID:{{c1::Monthly IVIg infusions}}{{c1::Antibiotic prophylaxis with TMP-SMX (Bactrim) against PJP}}Absolute: {{c1::haematopoietic stem c…
Published	10/08/2024	Ataxia Teleangiectasia is an AR disease caused by a mutation in the Ataxia Teleangiectasia Mutated Protein which normally fixes damaged DNA. As c…
Published	10/08/2024	Diagnosis of Ataxia Teleangiectasia includes:{{c1::Decreased levels of ATM protein}}Treatment:{{c1::Prophylactic antibiotics}}{{c1::Regular IVIg infus…
Published	10/08/2024	Wiskott-Aldrich syndrome is caused by a mutation in the WASP protein which causes a defect in cell cytoskeleton = T and B cells cannot form an immunol…
Published	10/08/2024	Wiskott-Aldrich syndrome: Diagnosis{{c1::Peripheral smear: thrombocytopenia}}{{c1::Flow cytometry: mutated protein}} {{c1::Sequencing for gene}}
Published	10/08/2024	Wiskott-Aldrich syndrome: Treatment (5){{c1::Prophylactic antibioticsIVIGPlatelet transfusionsSplenectomy to maintain platelet countHaematopoietic ste…
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