MCM 2-26 V2 Flashcards Preview

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Flashcards in MCM 2-26 V2 Deck (18)
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MHC antigen deficiencies

wont have CD4 or CD8 t cells = B cells will be crippled, get lots of common and opporunitistc infections

auto-somal recessive, various genes, lots of respiratory, common and opportunistic infections



IL2 receptor error, RAG error = wont get T cell receptors or antibody made by B-cells

will see diseases seen in aids (candidiasis), GFVD


Chronic Granulomatous Disease

NADPH oxidase mutation = phagocyte issues = cant make oxygen reactive species used by phags to kill. When phags cant kill they form granulomas

Increased susceptibility to catalase + bacteria that can break down the small amounts of H202 generated

impaired neutrophil function

chronic bacterial and fungal infections


Selective IgA Deficiency

no specific deficiencies, most common immunodeficiency. may be asymptomatic,

those who are symptomatic may have frequent respirartory and GI infections


X linked agammablobulinemia

No IgG antibody made
do not give live vaccines

Brutons tyrosine kinase

B cells blocked at pro-B stage

Recurrent bacterial infections


DiGeorge's Sundrome

deletion of 22q11.2 messes up head/neck/chest development

missing T cells
heart issues

unusual faces

absense of thymus and T-cells, need thymus transplant

GVHD is a problem, must only give irradiated blood products


Hyper IgM

without CD40L on t-cells, cant activate B-cells to class switch


Aids defining diseases

immunocompetent patients will not frequently get thrush, esophageal candidiasis, toxplasmosis

as cd4 decreases, these infections come at expected intervals


who should not get live vaccines?

anyone with combined humoral deficiency - AKA missing B cells or T cells


describe the common IL2 gamma chain defect

The IL2 gamma chain in interleuken2, invovled in signal transduction, is also involved in IL4, 7, 9, 15, 21 tranduction

Missing the common gamma chain causes failure of major cytokine receptors required to make T and B cells

Result - don’t make T and B cells.

Common gamma chain.

Missing big chunks of immune response - children very ill, cant make B or T cells


scids heredity?


x linked and auto recessive
75% male

bone marrow transplant to prevent early death


treatment of cancer vs others?

in cancer we want to boost immune response, generally in other conditions we want to dial it down.


oral yeast infection is a sign of

Weak CMI


consequence of lacking MHC1? MHC2?

missing mhc1 - cant educate CD8+ t cells
missing MHC2 - cant educate CD4+ t cells


missing the common gamma chain of IL2 receptor causes

failure of major cytokine receptors required to make T and B cells


combined immunodeficiency will often show

CBC often shows lymphopehnia or no lymphocytes at all


diagnosis of combined immunodeficiency

-complete blood count will show lymphopenia
-enumeration of specific lymphocyte subsets assists in diagnosis
-analysis of lymphocyte proliferation in response to mitogens, non-self HLA antigens in a mixed lymphocyte culture, specific antigens


treatment of combined immunodeficiency

needs bone marrow transplant

do not give live vaccine

treat with antibotics, antivirals, antifungals etc..

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