Callans. Flashcards by R B

Goodpastures

Type 2 hypersensitivity

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Neutropenia risk high is TPMT low

Azathioprine

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Recurrent infections with no neutrophils in FBC

Kostmann syndrome

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1 yo boy. Recurrent bacterial infections. Cd4 and cd8 T cells present. B cells absent, IgG, IgA, IgM absent.

Brutons x linked hypogammaglobulinaemia

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Infection with atypical mycobacterium. Normal fbc.

Ifn gamma receptor deficiency

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Bonds to microbial surface carbohydrates to activate the complement cascade in an immune complex independent manner

Mbl

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Binding of immune complexes to this protein triggers the classical pathway of complement activation

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PML

Mycophenolate mofetil

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Derived from monocytes and resident in peripheral tissues

Macrophages

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Recurrent respiratory tract infections, absent IgA, normal IgM and IgG

IgA deficiency

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Express receptors that recognise peptides usually derived from intracellukar proteins and expressed in hla class 1 molecules

Cd8 T cells

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Recurrent infections with hepatosplenomegalg and abnormal dihydrorhodamine test

Chronic granulomatous disease

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Exist within the bone marrow and develop from haemotopoietic stem cells

Pre B cells

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Lymphocytes that express inhibitory receptors capable of recognising hla class 1 molecules and have cytotoxic capacity

Nk cells

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Eczema

Type 1 hypersensitivity

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Osteoporosis

Prednisolone

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May be mediated by antibodies, complement components or acute phase proteins and facilitates phagocytosis

Opsonisation

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Inhibits T cell migration but may only be used in highly active remitting/ relapsing MS

Natalizumab (anti-a4 integrin)

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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

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Reduce risk of TB infection

BCG vaccination

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Chronic granulomatous disease

ifn Gamma

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Cleavage of this protein may be triggered via the classical, mbl it alternative pathways

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Subset of lymphocytes that express foxp3 and cd25

T reg cells

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Severe childhood onset SLE with normal levels of c3 and c4

C1q deficiency

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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

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

Callans. Flashcards

(67 cards)