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Immunology - Immunodeficiency Disorders (*Immunodeficiency Disorders = IDs…
Immunology - Immunodeficiency Disorders
*Immunodeficiency Disorders = IDs
*Hyper-IgM Hyperimmunoglobulin M Syndrome
all other immunoglobulins are low
X-linked recessive gene inheritance of CD20L
--> very rare that CD20+ antigen on B cell is mutated (auto recessive then)
Hyper-IgM Hyperimmunoglobulin M Syndrome Case
Clinical Case
Notes
:
note that Hyperimmunoglobulin M syndrome is where the T cells cannot communicate with B cells that produce antibodies to do class switchin
note that IgM antibodies are the most abundant
--> they are the default antibodies that B cells make
CD40L on T cells and CD20+ antigen on B cells allows Tcell --> B cell activation and antibody class switching
--> note that the class may switch, but the variable region where the antigen binds stays the same?
think WB 20 for B cells, also WB 40 --> for class switching
*B cell IDs
*T cell IDs
*Phagocyte disfunction IDs
*Chronic Granulamatous Disease = CGD
deficient in NADPH oxidase
--> needed in lysosomes fro making reactive oxygen species to kill bacteria that it eats
*T cell and B cell Immunodeficiency Disorders = IDs
*SCID
Severe combined immunodeficiency
*ADA = common cause of SCID
*SCID from ADA deficiency
affects BOTH T and B cells
1 more item...
Clinical Cases
Clinical Case
Clinical Case
Notes
:
note that
SCID Case
Clinical Case
Notes
:
note that SCID is severe deficiency of both T cells and B cells
they usually present in infancy, they are fine while they have their mother's immunity still in them, but as that wears off they usually get recurrent infections
--> big indication is recurrent candidiasis infections
--> this tells you they are immunocompromised
note in the above that all the electrolytes are normal, except that calcium was high --> why given?
--> Ca++ levels are an important differentiator between DiGeorge Syndrome
DiGeorge Syndrome has HYPO Calcemia and SCID has HYPER calcemia
--> think of SCID being so SEVERE that they feel the need to drink alot of milk and raise their CA++
--> think of DiGeorge as me = George being sick and not drinking any milk --> HYPO calcium --> weird
another key to SCID that is in this case is the diarrhea which is unique to SCID among the other immunodeficiencies
*Ataxia Telangectasia = HP Horcrux triad
A TRIAD
--> ataxic
--> Angiomas
--> low IgA (A G and E low)
*Wiskott-Aldrich ID
wasp WA-TER TRIAD
--> thrombocytopenia
--> eczema
--> recurrent RTI infections
*Hyper IgM CD40 ID
loss of CD40L leads to hyper IgM levels and lower of all other antibodies
--> since lost the CD40 needed for class switching to other GAMED antibodies
NOTES
:
most immuno- deficiencies have both T cell and B cell deficiencies
T cell deficiency
--> low CD3+ count (undifferentiated T cells)
B cell deficiency
--> Agammaglobulin
--> note that gamma globulins just refer to globulins that are heavy on gel electro.
--> immunoglobulins = antibodies made by B cells are agammaglobulins
*DIRTY USMLE
IMMUNO DEFICIENCIES
Sir Chediak Higashe SQUIRE
Sir Chediak Higashe SQUIRE
LYST of demands = LMNOP
LYST = the lysosomal protein that is mutated
LYST of demands = LMNOP
L = lymphohistiocytes
M = microtubule problems
N = Neuroloical symptoms
OP = phagolysosome formation problems
Pancytopenia
CRHONIC GRANULOMATOUS DISEASE
= CATALASE granuloma disease
Nitro blue test for
MYELO POx
