Flow cytometric screening in primary immune deficiencies. Melissa Dullaers 16/05/2013
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1 Flow cytometric screening in primary immune deficiencies Melissa Dullaers 16/05/2013
2 The immune system: defense against pathogens Barrier functions skin, mucosae, mucus, ciliae Reflexes coughing, sneezing, diarrhea Innate immunity complement, lyzozyme, lactoferrin, defensins phagocytes: neutrophils, macrophages, dendritic cells natural killer cells Adaptive immunity humoral immunity: B cells + immunoglobulins cellular immunity: CD4 + CD8 T cells 19/4/13 Melissa Dullaers 2
3 Innate immunity complement activation chemotaxis phagocytosis respiratory burst cytolyse natural killer cel complement Tumor cells neutrophil monocyte macrophage 19/4/13 Melissa Dullaers Virus-infected cell 3 K. Vermaelen
4 Adaptive Immunity: anti-bacterial; anti-fungal IL-17 Neutrophilic inflammation IL-6/IL-23 STAT3 Th17 (CD4) Th2 (CD4) Follicular helper T cell (Tfh) CD40 ICOS B-cel TLRs IL-12 Th1 (CD4) IFNγ antibodies Plasma cell opsonization FcR Macrophage recruitment + activation 4
5 Adaptive Immunity: anti-viral Neutralizing Abs IFNα/β Th2, Tfh TLRs CD8 Th1 CD40 CTL IFNγ perforin granzyme
6 Innate Immunity deficiencies Deficient phagocyte func1on PHOX (NADPH oxidase) defect: chronic granulomatous disease b2 integrin defect: Leukocyte adhesion deficiency: recurrent bacterial infecoons Shwachman- Diamond syndrome (SBDS protein) Complement disorders Factor H deficiency MBL deficiency 19/4/13 Melissa Dullaers 6
7 AdapOve immunity: combined B + T cell deficiencies Severe combined immuno- deficiency SCID: boy in the bubble (>20 genes eg RAG, IL7Ra, JAK3, ZAP70 ) Omenn syndrome MHC I/II deficiency
8 AdapOve immunity: immunoglobulin deficiencies Most prevalent: Common variable immune deficiency = acquired hypogamma- globulinemia (TACI, CD19, ICOS ) Hyper IgM syndrom (CD40, CD40L, AID) IgG subclass deficiency
9 Importance of flow cytometry Clinical spectrum of PID is very diverse Provide supporove data to largely clinical, radiological and family history MulOcolor flow allows evaluaoon of protein expression and/ or funcoon in different cell types of interest Although diagnosoc in some cases: geneoc tesong is necessary to confirm mutaoon
10 PIDs diagnosed by specific flow cytometry From Abraham Clinical Molecular Allergy 2011
11 First line flow panels for PID Lymphocyte panel: CD3, CD4, CD8, CD16, CD19, CD56, CD45 T cell development: CD3, CD4, CD8, CD45RO, CD45RA B cell development: CD45, CD10, CD19, CD21,CD24, CD27, CD38, IgD, IgM Regulatory T cells: CD3, CD4, CD8, CD45, Foxp3, CD25, CD127 Lymphocyte transformaoon test (LTT): CFSE, CD3, CD4, CD8, CD45 NK cell funcoon: Granzyme, perforin in rest IFNg, CD107 aher acovaoon (K562 or PMA/Ionomycin)
12 Case 1 (KI): 4 months old girl At hospitaliza1on: hepatospleomegaly raised transaminases fever neutropenia mild thrombocytopenia campylobacter jejuni è suspicion HLH 2 nd episode PneumocysOs carinii pneumonia CMV Rotavirus è suspicion SCID
13 3,16+56,45,4,19,8.fcs 10.7 <APC-A>: CD19 CD <PE-A>: CD16+56 CD <PE-Cy7-A>: CD <PerCP-Cy5-5-A>: CD45 <FITC-A>: CD3 <FITC-A>: CD3 <APC-H7-A>: CD8 T-cel uitrijping tube.fcs, subset 47.9 <PerCP-Cy5-5-A>: CD3, subset 27.3 <APC-A>: CD <FITC-A>: CD45 RA Naive 8.77 memory 84.7 <FITC-A>: CD45 RA Naive 51.1 memory 43.9 <PE-Cy7-A>: CD8 <PE-A>: CD45 RO <PE-A>: CD45 RO T-cel uitrijping repeat, subset 39.6 <PerCP-Cy5-5-A>: CD3, subset 35.1 <APC-A>: CD <FITC-A>: CD45 RA Naive 8.05 memory 82.9 <FITC-A>: CD45 RA Naive 39.9 memory 52.5 <PE-Cy7-A>: CD8 <PE-A>: CD45 RO <PE-A>: CD45 RO
14 ALPS + HLA DR expr buis.fcs 48.8 <FITC-A>: CD nont 59.9 <APC-A>: CD4 DN <APC-A>: CD4 DR <PE-Cy7-A>: CD8 DR <HV500-A>: CD45 <PE-A>: TCR ab <PE-Cy7-A>: CD8 <HV450-A>: HLA DR <HV450-A>: HLA DR ALPS + HLA DR repeat 47.5 <FITC-A>: CD <APC-A>: CD4 DN <APC-A>: CD4 DR <PE-Cy7-A>: CD8 DR <HV500-A>: CD45 <HV500-A>: CD45 <PE-Cy7-A>: CD8 <HV450-A>: HLA DR <HV450-A>: HLA DR
15 B-celuitrijping tube 1.fcs <HV500-A>: CD45 <PE-Cy7-A>: CD19 <FITC-A>: IgD IgD <APC-A>: IgM IgM <FITC-A>: IgD Naive B 90.2 Q MZ B 1.42 sw memory B 1.01 <PerCP-Cy5-5-A>: CD27 <APC-H7-A>: CD38 CD CD38loCD21lo CD <PE-A>: CD21 <APC-H7-A>: CD38 CD score CD <PE-A>: CD24 <APC-A>: CD10 CD10 transb 15.7 <PE-Cy7-A>: CD19
16 <HV500-A>: CD45 <APC-H7-A>: CD8 <FITC-A>: CFSE <FITC-A>: CFSE 36.7 <HV450-A>: CD 1.01 <APC-A>: CD # Cells 0 # Cells 1.59 <HV500-A>: CD45 <APC-H7-A>: CD8 <FITC-A>: CFSE <FITC-A>: CFSE Tetanus.fcs 35.3 <HV450-A>: CD3 FSC-SSC 0.57 <APC-A>: CD # Cells 0 # Cells 1.86 <HV500-A>: CD45 <APC-H7-A>: CD8 <FITC-A>: CFSE <FITC-A>: CFSE Candida.fcs 36.3 <HV450-A>: CD3 FSC-SSC <APC-A>: CD # Cells 0 # Cells 1.98 <HV500-A>: CD45 <APC-H7-A>: CD8 <FITC-A>: CFSE <FITC-A>: CFSE ConA.fcs 29.7 <HV450-A>: CD3 FSC-SSC 6.06 <APC-A>: CD # Cells 77.6 # Cells 88.6 <HV500-A>: CD45 <APC-H7-A>: CD8 <FITC-A>: CFSE <FITC-A>: CFSE PHA.fcs 33 <HV450-A>: CD3 FSC-SSC 12.9 <APC-A>: CD # Cells 65 # Cells 93.8
17 Case 2 (MC): 8 months old boy Hospitaliza1on: Frequent infecoons 1 st 6 months Difficult breathing Low Igs No thymus on CT scan è Suspicion SCID
18 3,16+56,45,4,19,8.fcs 10.5 <APC-A>: CD19 CD <PE-A>: CD16+56 CD <PE-Cy7-A>: CD <PerCP-Cy5-5-A>: CD45 <FITC-A>: CD3 <FITC-A>: CD3 <APC-H7-A>: CD8 T-cel uitrijping tube.fcs, subset 32.8 <PerCP-Cy5-5-A>: CD3, subset 25.2 <APC-A>: CD <FITC-A>: CD45 RA Naive 0 memory 99.6 <FITC-A>: CD45 RA Naive 3.88 memory 91.1 <PE-Cy7-A>: CD8 <PE-A>: CD45 RO <PE-A>: CD45 RO
19 B-celuitrijping tube 1.fcs <HV500-A>: CD45 <PE-Cy7-A>: CD19 <FITC-A>: IgD IgD <APC-A>: IgM IgM <FITC-A>: IgD Naive B 98.5 Q MZ B sw mem B <PerCP-Cy5-5-A>: CD27 <APC-H7-A>: CD38 CD CD38loCD21lo CD <PE-A>: CD21 <APC-H7-A>: CD38 CD <PE-A>: CD24 CD <APC-A>: CD10 CD10 transb 8.78 <PE-Cy7-A>: CD19
20 Case 3 (VT): 4 months old boy Hospitaliza1on: 3x sepsis (s. aureus, veillonella, coag- staph) Itchy erythrodermia Alopecia IntesOnal maladsorpoon è Suspicion Omenn syndrom
21 Case 4 (SC): 22 year old man At hospitaliza1on: overall malaise weight loss nights sweats fever hemoptoe è suspected for lymphoma upon RX, PET- CT, mediasonoscopy BUT: atypical mycobacterium kansasii infecoon è suspected for mendelian suscep1bility to mycobacterial disease
22 important prequels: sustained thrombocytopenia since 10 years fulminant verrucae vulgaris on feet
23 3, 4, 8, 16, 56, DR, CD <HV500-A>: CD45 <PerCP-Cy5-5-A>: CD3 non T 18.2 TCR <PE-A>: TCR alfa/beta <APC-H7-A>: CD4 CD DN 3.01 TCR <PE-Cy7-A>: CD8 <APC-H7-A>: CD4 CD4 DR <HV450-A>: DR <PE-Cy7-A>: CD8 DR <HV450-A>: DR non T <FITC-A>: CD16 <APC-A>: CD56 NK 25.2 <APC-A>: CD56 <HV450-A>: DR B c 27 T-cel uitrijping tube.fcs, subset 29.7 <PerCP-Cy5-5-A>: CD3, subset 85.1 <APC-A>: CD <FITC-A>: CD45 RA Naive 33.6 memory 63.7 <FITC-A>: CD45 RA Naive 47.4 memory 46.6 <PE-Cy7-A>: CD8 <PE-A>: CD45 RO <PE-A>: CD45 RO
24 B-celuitrijping tube 1.fcs <HV500-A>: CD45 <PE-Cy7-A>: CD19 <FITC-A>: IgD IgD <APC-A>: IgM IgM <FITC-A>: IgD Naive B 34.3 Q MZ B 29.8 sw mem B 22.2 <PerCP-Cy5-5-A>: CD27 <APC-H7-A>: CD38 CD CD38loCD21lo CD <PE-A>: CD21 <APC-H7-A>: CD38 CD CD <APC-A>: CD10 CD10 transb 4.78 <PE-A>: CD24 <PE-Cy7-A>: CD19
25 Lymphocyte transformaoon test: PHA, ConA, PWM: normal growth Tetanos toxoid: 0.2% (>3.0%) chemotaxis: low- normal Lymphocyte flow: Monocytes B + T cells DendriOc cells PaOent Control 19/4/13 Melissa Dullaers 25 K. Vermaelen
26
27 Case 5 (CD): 8 months old boy Hospitaliza1on: Bilateral bronchopneumonia and ooos media Recurrent gastroenterios + cysoos GastrointesOnal malabsorpoon Progressive worsening Liver failure, high ferrion, high solcd25 è Suspected familiar HLH
28 NK 250K 200K 150K 100K 50K K 100K 150K 200K 250K Ungated PID5 1+2u BFA_PMA 5ug+io.fcs Event Count: <HV500-A>: aqua K 100K 150K 200K 250K debris out PID5 1+2u BFA_PMA 5ug+io.fcs Event Count: K 200K 150K 100K 50K 0 alive PID5 1+2u BFA_PMA 5ug+io.fcs Event Count: <HV450-A>: CD45 <PerCP-Cy5-5-A>: CD PID5 1+2u BFA_PMA 5ug+io.fcs Event Count: <PE-A>: CD56 <FITC-A>: IFNg <APC-A>: CD107a NK PID5 1+2u BFA_PMA 5ug+io.fcs Event Count: 8253 NK 250K 200K 150K 100K 50K K 100K 150K 200K 250K Ungated PID5 1u+2u BFA_001_RPMI.fcs Event Count: <HV500-A>: aqua K 100K 150K 200K 250K debris out PID5 1u+2u BFA_001_RPMI.fcs Event Count: K 200K 150K 100K 50K <HV450-A>: CD45 alive PID5 1u+2u BFA_001_RPMI.fcs Event Count: <PerCP-Cy5-5-A>: CD <PE-A>: CD56 PID5 1u+2u BFA_001_RPMI.fcs Event Count: <FITC-A>: IFNg <APC-A>: CD107a NK PID5 1u+2u BFA_001_RPMI.fcs Event Count:
29 Activated Controle Patient PID <FITC-A>: IFNg <FITC-A>: IFNg <APC-A>: CD107a <APC-A>: CD107a <FITC-A>: IFNg <APC-A>: CD107a rest <FITC-A>: IFNg <APC-A>: CD107a <FITC-A>: IFNg <APC-A>: CD107a <FITC-A>: IFNg <APC-A>: CD107a
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