Table 1.

Clinical data of 8 patients with CHS

Patient Age at Diagnosis Current Age (y) Accelerated Phases BMTConsanguinity Family History Neurologic Manifestations
Age at Onset Severity Suspected Triggering Factor
1  5.5  5.5  ++  EBV  −  −  
 6.0*     −  
2  2.5 2.5  +  EBV  +  +  Low intellectual capacity (QD = 80)  
 22      +  
3  14  14  ++ ?  −  +  Mental retardation  
 16     −  
4  1  1.6  ++  EBV  +  Poor performance at school  
 14* 14    −  
5  1.3  1.3  ++  EBV  − −  
 1.5*     +  −  
6  7.8 0  −  −  −  −  
 10      −  
7  <1  2  ++  EBV  −  +  Low intellectual capacity (QD = 75)  
 11      
8  <1  1  ++  ?  +  +  
 1.4*     +  −  
Patient Age at Diagnosis Current Age (y) Accelerated Phases BMTConsanguinity Family History Neurologic Manifestations
Age at Onset Severity Suspected Triggering Factor
1  5.5  5.5  ++  EBV  −  −  
 6.0*     −  
2  2.5 2.5  +  EBV  +  +  Low intellectual capacity (QD = 80)  
 22      +  
3  14  14  ++ ?  −  +  Mental retardation  
 16     −  
4  1  1.6  ++  EBV  +  Poor performance at school  
 14* 14    −  
5  1.3  1.3  ++  EBV  − −  
 1.5*     +  −  
6  7.8 0  −  −  −  −  
 10      −  
7  <1  2  ++  EBV  −  +  Low intellectual capacity (QD = 75)  
 11      
8  <1  1  ++  ?  +  +  
 1.4*     +  −  
*

Died.

Relapse after bone marrow graft rejection.

Patients 5 and 6 are siblings.

BMT, bone marrow transplantation; CHS, Chediak–Higashi syndrome; EBV, Epstein–Barr virus.

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