Table 1.

NK Cell Activity and Lymphocyte Subpopulation of 5 FHL Patients at Onset

Patient No. Age/Sex Affected Siblings NK Activity (%)*CD3 (%) CD19 (%) CD56 (%)CD3/DR (%)CD4 (%) CD8 (%) Outcome
1  1 mo/M  +  NT  82.5   6.8   7.2 46.3̲ 63.8  11.4  Died of PD after BMT (13 mo)  
2  18 mo/F  19.0 (1.0̲) 77.9  10.3 12.8  63.0̲ 15.9̲ 65.3̲ Died of PD (3.5 mo)  
3  2 mo/M  1.0̲ 85.6   4.7  8.8  19.8̲ 57.7  28.1 Alive after CBSCT (+23 mo)  
4  17 mo/F  1.0̲ 59.4  29.2  9.1  14.5̲ 27.3  25.3 Alive after CBSCT (+35 mo)  
5  1 mo/M  9.0̲ 67.1  15.8 14.6   0.5  47.1  22.9  Died of PD (3 mo) 
Patient No. Age/Sex Affected Siblings NK Activity (%)*CD3 (%) CD19 (%) CD56 (%)CD3/DR (%)CD4 (%) CD8 (%) Outcome
1  1 mo/M  +  NT  82.5   6.8   7.2 46.3̲ 63.8  11.4  Died of PD after BMT (13 mo)  
2  18 mo/F  19.0 (1.0̲) 77.9  10.3 12.8  63.0̲ 15.9̲ 65.3̲ Died of PD (3.5 mo)  
3  2 mo/M  1.0̲ 85.6   4.7  8.8  19.8̲ 57.7  28.1 Alive after CBSCT (+23 mo)  
4  17 mo/F  1.0̲ 59.4  29.2  9.1  14.5̲ 27.3  25.3 Alive after CBSCT (+35 mo)  
5  1 mo/M  9.0̲ 67.1  15.8 14.6   0.5  47.1  22.9  Died of PD (3 mo) 

Abnormal values are underlined.

Abbreviations: NT, not tested; PD, progressive disease.

*

Normal range is 18% to 46%.19 

Normal range of CD3+/DR+ cells is 6.4 ± 4.8.19 

In patient no. 2, 19% of NK activity decreased to 1% after 1 month.

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