Immuno Flashcards
Atypical granulomatous infection in previously healthy patient
IFN gamma, IFNgamma receptor deficiency, IL-12, IL-12 receptor
Salmonella, atypical
Mycobacteria, TB and BCG.
Patients are unable to form granulomata
6 days every 3 weeks; give G-CSF
Cyclic neutropenia
X linked tyrosine kinase - BTK gene
Failed production of mature B cells, no antibodies, symptoms 3-6 months
Brutons agammaglobulinaemia
congenital neutropenia=NO NEUTROPHILS
Kostmann
Rheumatoid arthritis patient with mycobacterial infections
Iatrogenic - TNF antagonists
Recurrent infections with negative NBT and dihidrorhodamine tests
Chronic granulomatous disease
Young girl with normal B cells, normal CD8+ but absence of CD4+ (jaundice and hepatosplenomegaly). kid unwell by 3 months of age:
Bare Lymphocyte Syndrome BLS. IgG would be low
(Answer type 1 and 2 exist, but more common to have type 2 with absent CD4 compared to type 1 with absent CD8).
Young boy with normal B cells and absence of CD8+ and CD4+
Di George
Immune deficiency that has improved with age but not mum is concerned about delayed speaking and language
Di George
Normal B and T cells, high IgM but absence of IgA, IgE and IgG– Pneumocystis Cariini and failure to thrive.
Answer – CD40L deficiency – this is hyper-IgM. Presents in first years of life with recurrent bacterial infections. X-linked; can’t class switch so no IgA and IgG
Patient with recurrent pneumococcal infections and meningitisforming membrane attack complex) can predispose to infection from encapsulated organisms.
C5-C9 Deficiency
A 6-month old boy with severe failure to thrive and recurrent infections, early infant death…etc… his T cells were undetectable and B cells were NORMAL even if you think they should be lower
(Answer – X-linked SCID) Failure to thrive, and deficiency in IL-2.
SLE. Membraneous lymphogromelulonephritis
C1q deficieny.
Marker of antibody mediated rejection
C4d
Most important to match for rejection
HLA: DR>B>A
Hyper-acute rejection (ABO incompatibility)
Preformed Ab activates complement
Thrombosis and necrosis, prevented by cross matching properly
Can lead to development of post transplantation lymphoproliferative disease. PTLD is a proliferation (rapid increase) of lymphoid (immune) cells. It can develop in people who are taking immunosuppressive drugs to prevent rejection of an organ or an allogeneic (donor) bone marrow or stem cell transplant
calceurin inhibitors
Causes progressive multifocal leukoencephalopathy
mycophenolaTe myofetil T cells
Mechanism behind GVHD Graft versus host disease (GvHD)
○ Donor cells attacking host
○ Days-weeks, rash, N+V, bloody diarrhoea, abdo pain, jaundice
○ Prophylaxis: methotrexate/cyclosporine
○ Treat with steroids
Mechanism behind antibody mediated rejection
○ Antibodies attack vessels
○ Exposure -> proliferation + maturation of B cells ->
○ Effector phase - antibodies bind to graft endothelium
(->vasculitis), two weeks after the actual transplant
Mechanism behind cellular rejection
○ T cells, type IV reaction
○ TReat with T cell suppressors
○ Donor APC presenting antibodies to recipient
○ Cellular infiltrate
Diffuse systemic scleroderma
scl-70 topoisomerase
Limited cutaneous scleroderma
anti-centromere
Anti phospholipid syndrome
anti-cardiolipin
epistaxis and haematuria
Wegener’s granulomatosis c-ANCA
Serum sickness - Type X reaction?
III reaction, following antibiotics, symptoms take 7-12h to develop
dry eyes and mouth antibodies
anti-Ro, anti-La
weight loss, anti TTG antibodies, anti-endomysial antibodies, steatorrhoea
Coeliac
CREST
Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia
SLE
anti ds DNA
Pernicious anaemia
anti parietal cell > anti intrinsic factor
heamoptysis and haematuria
goodpasture, anti-collagen membrane IV
How to monitor the activity of SLE?
C3 & C4 levels
How to monitor a patient with HIV?
CD4 T lymphocyte cell count to assess immune function and plasma HIV RNA (viral load) to assess level of HIV viremia.
How to monitor Autoimmune Hepatitis?
Anti-SM
FAS pathway
REMEMBER FAS IN THE ALPS: ALPS syndrome, auto-immune lymph-proliferative syndrome, mutation in the FAS gene which steers lymphoproliferation of the immunce cells. ALPS is characterized by the production of an abnormally large number of lymphocytes (lymphoproliferation). Accumulation of excess lymphocytes results in enlargement of the lymph nodes (lymphadenopathy), the liver (hepatomegaly), and the spleen (splenomegaly). FAS drives high lymphocyte proliferation, thus BIG BIG ALPES of hepatmogelay, SPLENOMGALY, SPLENOMEGALY
Mentioned MEFV gene, periodic abdominal pain and ascites.
Familial Mediterranean Fever
abnormal production of IL-1 and the attack of the pleura surfaces
Familial Mediterranean Fever
Nod2/CARD15
Crohns Disease
Single gene mutation involving FOXp3 resulting in the abnormality of Treg cells (Which normally regulate T-helper cell function)
IPEX – (Immune dysregulation polyendocrinopathy enteropathy an X-lined) is caused by
Autoimmune Polyendocrinopathy Syndrome Type 1 - AIRE mutation.
2/3 components: (regulatory trush) chronic mucocutaneous candidiasis, hypoparathyroidism adrenocortical insufficiency Treg cell defect.
Hereditary angioedema. A patient with some description [can’t remember details] of C1 inhibitor deficiency – she was still able to talk
C1 inhibitor deficiency C1 inhibitors, which are responsible for maintaining proper balance (homeostasis) in the complement system (specifically, keeping the C1 part of the classical complement pathway from being overactive). C1q esterase needed
When they eat cherries, apples, pears and hazelnuts, they get itchy mouths
Acute Angiodema/ Oral Allergy Syndrome
Hypertensive and diabetic, with angioedema
ACE inhibitors
Woman with flushed face, problems breathing that has happened multiple times with enlarged liver
carcinoid
A patient with some rash etc… after eating.
H1 oral antihistamines
A patient with some rash and wheeze etc
IM adrenaline
Cytokine responsible for promoting antibody synthesis
IgE
Main cellular source of histamine
Mast cell
How do you measure mast cell degranulation
Mast cell tryptase levels
Chemokine responsible for promoting eosinophil growth
IL5
Secrete Igs, cytokines, present antigens
Present antigens; migrate to lymph nodes when activated
Dendritic
CD8+
Cytotoxic - MHC I
In its immature form it is specialized to phagocyte pathogens, and in its mature form it is specialized to present to other cells
Monocyte (macrophages)
Monocytes resident in peripheral skin cells
Langerhans cell
Connective Tissue
Histiocyte
Cells that express Foxp3 and CD25
Treg
Differentiated B cells that produce immunoglobulins
The effect or cells
Responsible for the killing of cancerous cells and are inhibited by MHC-I –
NK cells
The multimetric immune modulator
ISCOM
Can be used for RA depletes B-cells
Anti-CD20 Rituximab,
Antibody against IL6 receptor
Used in caslteman’s, RA if not responding to methotrexate, sulfasalazine
Pooled Ig used in the treatment
CVID
Prophylaxis of allograft rejection, IV before + after Antibody against
IL-2Ralpha chain of CD25 Basiliximab
Inhibits T-cell migration and may only be used against highly active remitting/relapsing MS
Anti Alpha-4 integrin Natulizumab
Treatment options include; inhibition of IL-12/23, TNF-alpha and IL17a
Psoriasis
Treatment options include; inhibition of IL-6, TNF-alpha and depletion of B-cells
Rheumatoid arthritis
Antiproliferative agent - B>T cells affected) Alkylates guanine base of DNA, Damages
Cyclophosphamide
IFNgamma
Chronic granulomatous disease
Used to treat malignant melanoma, involved in T cell checkpoints
ANTI-PD1
Used to prevent transplant rejection, by blocking T cell proliferation and activation
mycophenolate myofetil
Vaccine that eradicated small pox
Vaccinia
Antibody to p40 subunit of IL12 and IL23
Ustekinumab
Antiproliferative agent - B>T cells affected
Cyclophosphamide
Used to treat malignant melanoma, involved in T cell checkpoints
Pemrolizumab (ANTI-PD1)
Hepatitis C, Hepatitis B, Kaposi’s sarcoma
Interferon alpha:
Interferon beta
Relapsing MS
influenza A only, targets M2 channel
Amantadine
NA inhibitors
Oseltamivir, zanamivir, sialic acid
