Table 3

Clinical and immunologic outcomes of hematopoetic SCT in patients with primary immunodeficiency/immunodysregulatory syndromes complicated by LPD

No.DiagnosisFollow-up after BMT, yLatest immunologyRequired Ig replacementLatest chimerism (mononuclear cells)Latest chimerism (granulocytes)Overall outcome
1 Undefined CID 7.3 Normal PHA and LSS. Low IgA. Poor Ab responses. Yes Mixed high-level Mixed low-level Alive and well. 
2 WAS 5.4 Normal PHA response, LSS and Ig. Poor pneumococcal response. Yes Full Full Alive. Recurrent infections, including pneumococcal sepsis. Improved on restarting IVIG. 
3 IE 5.3 Normal PHA response, LSS, Ig, and Ab responses. No Mixed high-level Mixed high-level Alive and well. 
4 Undefined CID 5.2 Normal PHA response, LSS, Ig,* and Ab responses. No Full Full Alive and well. 
5 ALPS 3.2 Normal PHA response, LSS, Ig, and Ab responses. No Full Full Alive and well. 
6 CHH 2.1 Normal PHA response, LSS, Ig,* and Ab responses. No Full Full Alive and well. 
7 XLP 1.4 Normal PHA response, LSS and Ig.† No Full Full Alive and well. 
8 DALD 1.0 Normal PHA response. Low B and CD4 cells.† No Full Full Alive and well. 
No.DiagnosisFollow-up after BMT, yLatest immunologyRequired Ig replacementLatest chimerism (mononuclear cells)Latest chimerism (granulocytes)Overall outcome
1 Undefined CID 7.3 Normal PHA and LSS. Low IgA. Poor Ab responses. Yes Mixed high-level Mixed low-level Alive and well. 
2 WAS 5.4 Normal PHA response, LSS and Ig. Poor pneumococcal response. Yes Full Full Alive. Recurrent infections, including pneumococcal sepsis. Improved on restarting IVIG. 
3 IE 5.3 Normal PHA response, LSS, Ig, and Ab responses. No Mixed high-level Mixed high-level Alive and well. 
4 Undefined CID 5.2 Normal PHA response, LSS, Ig,* and Ab responses. No Full Full Alive and well. 
5 ALPS 3.2 Normal PHA response, LSS, Ig, and Ab responses. No Full Full Alive and well. 
6 CHH 2.1 Normal PHA response, LSS, Ig,* and Ab responses. No Full Full Alive and well. 
7 XLP 1.4 Normal PHA response, LSS and Ig.† No Full Full Alive and well. 
8 DALD 1.0 Normal PHA response. Low B and CD4 cells.† No Full Full Alive and well. 

LSS indicates lymphocyte subsets; PHA, proliferative response to phytohemaglutinin; and Ig, immunoglobulin level.

*

Except for low IgA level.

†

Antibody responses not yet assessed.

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