Callans. Flashcards
Goodpastures
Type 2 hypersensitivity
Neutropenia risk high is TPMT low
Azathioprine
Recurrent infections with no neutrophils in FBC
Kostmann syndrome
1 yo boy. Recurrent bacterial infections. Cd4 and cd8 T cells present. B cells absent, IgG, IgA, IgM absent.
Brutons x linked hypogammaglobulinaemia
Infection with atypical mycobacterium. Normal fbc.
Ifn gamma receptor deficiency
Bonds to microbial surface carbohydrates to activate the complement cascade in an immune complex independent manner
Mbl
Binding of immune complexes to this protein triggers the classical pathway of complement activation
C1
PML
Mycophenolate mofetil
Derived from monocytes and resident in peripheral tissues
Macrophages
Recurrent respiratory tract infections, absent IgA, normal IgM and IgG
IgA deficiency
Express receptors that recognise peptides usually derived from intracellukar proteins and expressed in hla class 1 molecules
Cd8 T cells
Recurrent infections with hepatosplenomegalg and abnormal dihydrorhodamine test
Chronic granulomatous disease
Exist within the bone marrow and develop from haemotopoietic stem cells
Pre B cells
Lymphocytes that express inhibitory receptors capable of recognising hla class 1 molecules and have cytotoxic capacity
Nk cells
Eczema
Type 1 hypersensitivity
Osteoporosis
Prednisolone
May be mediated by antibodies, complement components or acute phase proteins and facilitates phagocytosis
Opsonisation
Inhibits T cell migration but may only be used in highly active remitting/ relapsing MS
Natalizumab (anti-a4 integrin)
Severe recurrent infections from 3 months, cd4 and cd8 T cells absent, nk cells absent, B cells present, igs low. Normal facial features and cardiac echocardiogram
X linked SCID
Reduce risk of TB infection
BCG vaccination
Chronic granulomatous disease
ifn Gamma
Cleavage of this protein may be triggered via the classical, mbl it alternative pathways
C3
Subset of lymphocytes that express foxp3 and cd25
T reg cells
Severe childhood onset SLE with normal levels of c3 and c4
C1q deficiency
Single gene mutation involving FOXp3 resulting in abrmonality of T reg cells
IPEX (immune dysregulation, polyendocrinopathy, enteropathy, x linked)
Is mediated by toll like receptors which recognise pathogen associated molecular patterns
Pattern recognition