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
Young adult with chronic infection with mycobacterium marinum
IFN gamma receptor deficiency
Site of deletion of T cells with innaprorpaitely high or low affinity for HLA molecules and of maturation of T cells into cd4+ or cd8+ cells
Thymus
Treatment options include inhibition if IL6, TNF alpha and depletion of B cells
Rheumatoid arthritis
Post transplant lymphoproliferative disorder
EBV specific CD8 T cells
Recurrent bacterial infections in a child, episode of pneumocystis pneumonia, high IgM, absent IgA and IgG
X linked hyper IgM syndrome due to cd40 ligand mutation
Are generated rapidly following antigen recognistion and are not dependent on cd4 T cell help
IgM secreting plasma cells
Recurrent infections when receiving chemotherapy but previously well
MBL deficiency
Include both the bone marrow and thymus; sites of b and T cell development
Primary lymphoid organs
Area within secondary lymphoid tissue where B cells proliferate and undergo affinity maturation and isotope switching
Germinal centre
Meningococcus meningitis with fhx of sibling dying of the same condition
C7 deficiency
Antibody specific for cd25 which inhibits T cell activation and is used to prevent rejection
Basiliximab (anti il2 receptor)
Recurrent infections in childhood, abnormal facial features, congenital heart disease, normal B cells, low T cells, low igA and igG
DiGeorge syndrome
Cell dependent on the presence of cd4 T cell help for generation
IgG secreting plasma cells
Depleted B cells and is effective in treatment of B cell lymphomas and rheumatoid arthritis
Rituximab (anti cd20)
Infertility
Cyclophosphamide
Metastatic melanoma
Pembrolizumab (anti PD1)
Recurrent infections with high neutrophil count on fbc but no abcess formation.
Leukocyte adhesion decifiency
Immunosuppressed VZ seronegarive indicidual after chicken pox exposure
Varicella zoster immunoglobulin.
May be mediated by bacteriocidal enzymes such as lysozyme
Non oxidative killing
Treatment options include inhibition of IL12/23, TNF alpha and IL17A
Psoriasis
Single gene mutation involving MEFV and affective the inflammasome complex, resulting in recurrent episodes of serositis
familial Mediterranean fever
Part of the final common pathway resulting in the generation of the membrane attack complex
C9
Bonds to CD80 and CD86 on antigen-presenting cells and inhibits T cell activation and is effective in Rheumatoid arthritis
Abatacept (VTLA4-ig fusion protein)
X Linked scid
Bone marrow transplantation
Subset of cells that express cd4 and secrete ifn gamma and il2
Th1 cells
Carries lymphocytes from lymph nodes back to the blood circulation
Thoracic duct
Nephrotixicity
Tacrolimus
Multiple sclerosis
Type 4 hypersensitivity
Polymorphonuclear cells capable of phagocytosing pathogens and killing by oxidative and non oxidative mechanisms
Neutrophils
Divalent antibody present within mucous which helps provide a constitutive barrier to infection
IgA
Immature cells are adapted for pathogen recognition and uptake whilst mature cells are adapted for antigen presentation to prime T cells
Dendritic cells
Membranoproliferative neohritis and bacterial infections
C3 deficiency with presence of nephritic factor
Play an important role in promoting germinal centre reactions and differentiation of B cells into igg and iga secreting plasma cells
T follicular helper (tfh) cells
SLE
Type 3 hypersensitivity
6 month baby with two recent serious bacterial infections. T cells present - but only cd8+ population. B cells present. IgM present but igG low
Bare lymphocyte syndrome type 2
Mutation within the Fas pathway associated with lymphocytosis, lymphomas and auto immune cytopenias
Auto immune lymphoproliferatice syndrome (ALPS)
Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis
Tocilizumab (anti il6 receptor)
Describes killing mediated by reactive oxygen species generates by action of the nadph complex
Oxidative killing
Adult with bronchiectasis, recurrent sinusitis and development of atypical SLE
Common variable immunodeficiency
Treatment options include inhibition if RANK ligand
Osteoporosis
X linked hyper IgM syndrome
Human normal immunoglobulin
