Erratum to: J Clin Immunol (2016)
DOI 10.1007/s10875-016-0232-2
The original version of this article unfortunately contained mistakes in Table 1 and author’s contribution. The correct version is presented on the next page.
Table 1.
Clinical features and laboratory values for patients, demonstrating profound CD4-lymphopenia and reduced lymphoproliferative responses
| Featurea | P1 | P2 | P3 | Normal rangec |
|---|---|---|---|---|
| Clinical features | ||||
| Eczema & skin infections | + | + | − | |
| Recurrent sinopulmonary infection | + | + | − | |
| Oral candidiasis | − | + | − | |
| Cryptosporidiosis | + | + | + | |
| Viral infections: | ||||
| Molluscum contagiosum | + | + | + | |
| Recurrent VZV | − | − | + | |
| Severe 1° HSV | + | + | − | |
| Chronic EBV viremia | + | + | + | |
| EBV LPD | + | + | − | |
| Flow cytometric immunophenotyping (absolute cell counts per μl except where % indicated) | ||||
| CD3+ T cells | 815 | 850 | 921 | 1400–8000 |
| CD4+ T cells | 200 | 268 | 368 | 900–5500 |
| bnaive CD4+ T cells | 0 | 17 | 18 | |
| CD8+ T cells | 512 | 592 | 523 | 400–2300 |
| bnaïve CD8+ T cells | 16 | 17 | 28 | |
| TCR-γδ+ (% of T cells) | 28 | 7 | 15 | |
| HLA-DR+ (% of T cells) | 84 | 77 | 62 | |
| CD16+/CD56+ NK cells | 192 | 147 | 298 | 100–1400 |
| CD19+ B cells | 439 | 897 | 1355 | 600–3100 |
| CD27+IgD− (% of B cells) | 2 | 3 | 5 | |
| Lymphocyte proliferation assay | ||||
| PHA patient (cpm) | 11,326 | 34,343 | 67,254 | |
| PHA control (cpm) | 148,110 | 122,215 | 101,164 | |
| SI patient | 82 | 29 | 94 | |
| SI control | 445 | 407 | 349 | |
| Immunoglobulins | ||||
| IgG | 18.3 | 28.8 | 17.8 | 3.0–13.3 g/L |
| IgA | 2.2 | 4.47 | 0.7 | 0.3–1.29 g/L |
| IgM | 0.6 | 1.13 | 0.9 | 0.43–1.9 g/L |
| Pneumococcal ab | <3 | 17 | 88 | 20–200 mg/L |
| Tetanus ab | 0.88 | 1.55 | 0.49 | 0.1–10 IU/ml |
| Hemophilus B ab | NT | >9.0 | NT | 1.0–20 mg/L |
VZV Varicella zoster virus, HSV Herpes simplex virus, EBV Epstein Barr Virus, LPD lymphoproliferative disease, PHA phytohemagglutinin, cpm counts per minute, SI stimulation index, ab specific antibody, NT not tested
aLaboratory evaluation was performed at 5.5 years (P1), 3.4 years (P2), 1.7 years (P3)
bNaïve T cells were defined as CD3 + CD27 + CD45RA+ and as either CD4+ or CD4−
cNormal ranges are age matched to P3
Footnotes
Tarana Singh Dang and Joseph D. P. Willet contributed equally to this work.
The online version of the original article can be found at 10.1007/s10875-016-0232-2.
