MEDICAL RECORD # (PATIENT ID) CLIENT ID/WARD CLIENT/NAME WARD DLMP Rochester DATE COLLECTED 8/23/2015 3:00 AM DATE RECEIVED 8/24/2015 3:14 PM

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1 Performing Site: Mayo Clinic Laboratories - Rochester Main Campus 200 First Street SW, Rochester MN William G. Morice, II, M.D., Ph.D. Phone: CLIENT TCR Spectratyping, B Result Name Result Flag Units Reference Values Family 2 Diversity Ratio 2 % >=2 Family 3-1 Diversity Ratio 0 % >=0 Family 4 Diversity Ratio 3 % >=3 Family 5 Diversity Ratio 2 % >=2 Family 6 Diversity Ratio 5 % >=5 Family 7 Diversity Ratio 11 % >=11 Family 9 Diversity Ratio 3 % >=3 Family 10 Diversity Ratio 5 % >=5 Family 11 Diversity Ratio 1 % >=1 Family 12 Diversity Ratio 3 % >=3 Family 13 Diversity Ratio 3 % >=3 Family 14 Diversity Ratio 2 % >=2 Family 15 Diversity Ratio 2 % >=2 Family 16 Diversity Ratio 2 % >=2 Family 18 Diversity Ratio 3 % >=3 Family 19 Diversity Ratio 2 % >=2 Family 20-1 Diversity Ratio 3 % >=3 Family 24-1 Diversity Ratio 3 % >=3 Family 25 Diversity Ratio 1 % >=1 Family 27 Diversity Ratio 0 % >=0 Family 28 Diversity Ratio 3 % >=3 PATIENT NAME: 1

2 CLIENT Result Name Result Flag Units Reference Values Family 29 Diversity Ratio 3 % >=3 Family 30 Diversity Ratio 3 % >=3 Reviewed by Roshini Sarah Abraham, Ph.D., D(ABMLI), FAAAAI Interpretation Interpretation: CD3 = 1200 cells/ul CD4 = 800 cells/ul CD8= 400 cells/ul Normal TCR Vb repertoire diversity with polyclonal representation with Gaussian distribution in all families. This result is consistent with normal TCR Vb repertoire distribution for age and correlates with the normal T cell counts and thymic function (CD4RTE result reported separately). This result supports thymic-derived T cell reconstitution post-allogeneic HCT and shows significant repertoire recovery compared to the previous analysis performed at 3 months post-hct. Clinical Context: s/p allo-hct for GATA2 deficiency on 8/23/2104. Sample information: This sample is 9 months post-baseline analysis and is a post-transplant sample. Reference Value Message: Images of the actual spectratypes will be provided on physician request to the Laboratory Director. Method: This is a semi-quantitative assay performed by fragment-length analysis for TCR Vb CDR3 fragments. Analysis includes visual assessment of the spectratype and semi-quantitative measures of peak numbers (not reported) and the diversity ratio (DR) for 23 TCR Vb families (or sub-families). Diversity ratios are calculated as the ratio of total peak numbers for a specific family for a patient over the total peak numbers for the same patient. Subfamily diversity ratios are consolidated into a single diversity ratio for the entire specific TCR Vb family. Consensus primers, specific to each family, were used to collect TCR Vb CDR3 fragment length data for all subfamilies within each family. Those families indicated by -1 contain only a single subfamily that were collected using a single consensus primer. However, for certain families (6, 7 and 10) a consensus primer was not sufficient to collect all PATIENT NAME: 2

3 CLIENT subfamilies. In these cases, separate primers were used for each subfamily (6-4, 7-2, 7-3, 7-4 and 10-1) and then the data from the different sets of primers for a specific family were combined. Disclaimer: Laboratory developed test. PATIENT NAME: 3

4 CLIENT PATIENT NAME: 4

5 CLIENT PATIENT NAME: 5

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