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. 2016 Mar 3;36:336–337. doi: 10.1007/s10875-016-0248-7

Erratum to: Defective Leukocyte Adhesion and Chemotaxis Contributes to Combined Immunodeficiency in Humans with Autosomal Recessive MST1 Deficiency

Tarana Singh Dang 1,#, Joseph D P Willet 1,#, Helen R Griffin 2, Neil V Morgan 3, Graeme O’Boyle 1, Peter D Arkwright 4, Stephen M Hughes 4, Mario Abinun 1,5, Louise J Tee 3, Dawn Barge 6, Karin R Engelhardt 1, Michael Jackson 5, Andrew J Cant 1,5, Eamonn R Maher 3,7, Mauro Santibanez Koref 2, Louise N Reynard 1, Simi Ali 1, Sophie Hambleton 1,5,
PMCID: PMC4970337  PMID: 26941167

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.


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