Table 2

Hematologic and immunologic characteristics

Positive/evaluable patients (%)
Bone marrow examination  
Normal 37/108 (34.3) 
Granular hypoplasia 16/108 (14.8) 
Late maturation arrest 33/108 (30.6) 
Increase cellularity 22/108 (20.3) 
Marrow cytogenetic analysis available 60/108 (55.6) 
Lymphocyte counts (109/L), median [IQR] 1.4 [1.11-1.9] 
Lymphopenia 34/108 (31.5) 
Monocytopenia 8/108 (7.4) 
Monocytosis 5/108 (4.6) 
CD4/CD8 ratio 1.52 [1.21-2.10] 
Gammaglobulin level (g/L), median [IQR] 10.9 [9.9-13.1] 
IgG level (g/L), median [IQR] 11.8 [9.6-13.2] 
Hypergammaglobulinemia (%) 28/96 (29.2) 
Neutrophil autoantibodies 23/66 (34.8) 
 CD16 specificity 10/23 (43.5) 
 Unknown specificity 13/23 (56.5) 
T-cell clone (%) 6/47 (12.8) 
Biological autoimmunity  
 ANAs 20/82 (24.1) 
 Anti-dsDNA 9/62 (14.3) 
 Anti-SSA/Ro 1/45 (2.2) 
 Anti-SSB/Lo 0/42 (0) 
 Rheumatoid factor 10/64 (15.4) 
 ANCA 4/22 (17.4) 
 DAT 0/31 (0) 
 Other autoantibodies present 9/108 (8) 
 Overall (at least 1 positive) 39/83 (47) 
Positive/evaluable patients (%)
Bone marrow examination  
Normal 37/108 (34.3) 
Granular hypoplasia 16/108 (14.8) 
Late maturation arrest 33/108 (30.6) 
Increase cellularity 22/108 (20.3) 
Marrow cytogenetic analysis available 60/108 (55.6) 
Lymphocyte counts (109/L), median [IQR] 1.4 [1.11-1.9] 
Lymphopenia 34/108 (31.5) 
Monocytopenia 8/108 (7.4) 
Monocytosis 5/108 (4.6) 
CD4/CD8 ratio 1.52 [1.21-2.10] 
Gammaglobulin level (g/L), median [IQR] 10.9 [9.9-13.1] 
IgG level (g/L), median [IQR] 11.8 [9.6-13.2] 
Hypergammaglobulinemia (%) 28/96 (29.2) 
Neutrophil autoantibodies 23/66 (34.8) 
 CD16 specificity 10/23 (43.5) 
 Unknown specificity 13/23 (56.5) 
T-cell clone (%) 6/47 (12.8) 
Biological autoimmunity  
 ANAs 20/82 (24.1) 
 Anti-dsDNA 9/62 (14.3) 
 Anti-SSA/Ro 1/45 (2.2) 
 Anti-SSB/Lo 0/42 (0) 
 Rheumatoid factor 10/64 (15.4) 
 ANCA 4/22 (17.4) 
 DAT 0/31 (0) 
 Other autoantibodies present 9/108 (8) 
 Overall (at least 1 positive) 39/83 (47) 

Anti-SSA/Ro, anti-Sjogren's-related antigen A/Ro; anti-SSB/La, anti-Sjogren's-related antigen B/Lupus La; ANCA, antineutrophil cytoplasmic antibodies; DAT, direct antiglobulin test

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