Signaling impairments in maternal T cells engrafted in an infant with a novel IL-2 receptor [gamma] mutation

• Published in: Journal of allergy and clinical immunology Vol. 135; no. 4; p. 1093
• Main Authors: Khanolkar, Aaruni, Wilks, Jeffrey D, Jennings, Lawrence J, Davies, Jessica L, Zollett, Joshua A, Lott, Loren L, Fullmer, Erin R, Bensen, Nicolas E, Carlson-Leuer, Katrin M, Tse, William T, Fuleihan, Ramsay L
• Format: Journal Article
• Language: English
• Published: St. Louis Elsevier Limited 01.04.2015
• Subjects: Immune system; Lymphocytes; Medical research; Mutation
• ISSN: 0091-6749; 1097-6825
• DOI: 10.1016/j.jaci.2015.02.012

Date Patient Total cells in whole blood Purified myeloid cells Purified B cells Purified T cells June 2013 96 51 ND >98[low *] July 2013 97 96 >98 >98 August 2013 94 92 95 >98 September 2013 89 81 89 >98 October 2013 88 72 85 >98 November 2013 91 81 92 >98 December 2013 97 95 97 >98 January 2014 >98 98 ND >98 March 2014 >98 >98 >98 >98 June 2014 >98 >98 >98 >98 Table II Posttransplantation chimerism analyses Numeric values denote the percentage of cells carrying fully informative alleles derived from the stem cell donor (older female sibling).ND, Not done. Immunoglobulin isotype Before transplantation (mg/dL) 6 mo After transplantation (mg/dL) Patient values Age-associated normal ranges (>124 and <337 d) Patient values Age-associated normal ranges (>337 and <690 d) Â Serum concentration (kU/L) Serum concentration (kU/L) IgM 5.6 33-97 127 41-164 IgG <33.3 206-676 1150 331-1165 IgA <6.7 8-67 59 14-105 IgE <2 <16.4 2.9 <29.2 Table E1 Pretransplantation and posttransplantaton immune assessment of the patient: serum immunoglobulin concentration Parameter Before transplantation (cells/[micro]L of blood) 6 mo After transplantation (cells/[micro]L of blood) Patient values Normal ranges (5th and 95th percentiles [ages >124 and <337 d]) Patient values Normal ranges (5th and 95th percentiles [ages >337 and <690 d]) Â Lymphocytes (%) Lymphocytes (%) CD3+ 1267 2889-7995 3169 2207-8192 (CD3+)CD8+ 865 946-2791 1089 750-3749 (CD3+)CD4+ 363 1786-5141 1868 1089-4552 (CD3-)CD16+CD56+ 27 174-1102 380 182-1581 (CD3-)CD19+ 627 858-3774 1246 704-2711 CD3+HLA-DR+ 45 3-9 6 4-8 Table E2 Pretransplantation and posttransplantation immune assessment of the patient: absolute lymphocyte counts Mitogen Before transplantation (SI) 6 mo After transplantation (SI) Normal cutoff Patient Healthy control subject Patient Healthy control subject PHA (10 [micro]g/mL) 4.1 349.7 791 254 >94.0 PHA (1.25 [micro]g/mL) 1.4 378.2 216 110 >21.7 PWM (15.62 [micro]g/mL) 3.7 40.8 49 45 >10.5 PWM (0.78 [micro]g/mL) 4.5 83.0 52 35 >8.0 ConA (100 [micro]g/mL) 1.6 332.4 141 166 >8.6 ConA (50 [micro]g/mL) 2.0 288.3 772 261 >75.7 ConA (12.5 [micro]g/mL) 5.4 264.6 300 65 >25.6 Table E3 Pretransplantation and posttransplantaton immune assessment of the patient: tritiated thymidine incorporation assay ConA, Concanavalin A; PWM, pokeweed mitogen; SI, stimulation index.