Clinical Data
| Patient . | Sex . | Age at Onset . | Age at Study . | Platelets . | Hb (g/dL)* . | ANC . | Serum IgG (mg/dL)* . | DN T cells† (μL) . | LN‡ . | Spleenρ . | LAC . | ANA . | a-DNA . | Clinical Status at Study . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | . | . | . | (×103/μL)* . | . | (×103/μL)* . | . | . | . | . | . | . | . | . |
| 1 | M | 13 yr | 22 yr | 5-480 | 7.4-12.8 | 0.18-4.5 | 1,150-1,600 | 5% (130) | +++ | +++ | + | + | − | Neutropenia, mild thrombocytopenia, no therapy |
| 2 | M | 7 yr | 16 yr | 2-160 | 8.2-13.5 | 1.8-8.6 | 1,500-2,410 | 4% (100) | +++ | + | + | + | − | Severe thrombocytopenia, in therapy with methyl-PDN 10/mg/kg every 4 wk |
| 3 | F | 8 mo | 8 yr | 91-480 | 7.4-11.5 | 0.17-4.5 | 2,120-3,510 | 13% (140) | + | ++++ | + | + | − | Mild anemia and thrombocytopenia, no therapy |
| 4 | M | 4 yr | 14 yr | 5-924 | 5.5-14.5 | 0.38-7.8 | 345-640 | 5% (100) | +++ | ++ | + | − | − | Severe neutropenia and thrombocytopenia, in therapy with PDN 1 mg/kg/d |
| 5 | M | 3 yr | 8 yr | 8-167 | 11.6-14.1 | 1.0-6.8 | 550-922 | 9% (150) | +++ | ++ | + | − | − | In remission, no therapy |
| 6 | M | 4 mo | 3 yr | 17-267 | 7.3-12.7 | 0.7-5.6 | 408-760 | 4% (120) | + | +++ | + | − | − | In remission, no therapy |
| 7 | M | 2 yr | 3 yr | 45-168 | 9.7-11.8 | 1.6-4.7 | 670-795 | 2% (53) | + | +++ | + | − | − | Mild thrombocytopenia, no therapy |
| Patient . | Sex . | Age at Onset . | Age at Study . | Platelets . | Hb (g/dL)* . | ANC . | Serum IgG (mg/dL)* . | DN T cells† (μL) . | LN‡ . | Spleenρ . | LAC . | ANA . | a-DNA . | Clinical Status at Study . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | . | . | . | (×103/μL)* . | . | (×103/μL)* . | . | . | . | . | . | . | . | . |
| 1 | M | 13 yr | 22 yr | 5-480 | 7.4-12.8 | 0.18-4.5 | 1,150-1,600 | 5% (130) | +++ | +++ | + | + | − | Neutropenia, mild thrombocytopenia, no therapy |
| 2 | M | 7 yr | 16 yr | 2-160 | 8.2-13.5 | 1.8-8.6 | 1,500-2,410 | 4% (100) | +++ | + | + | + | − | Severe thrombocytopenia, in therapy with methyl-PDN 10/mg/kg every 4 wk |
| 3 | F | 8 mo | 8 yr | 91-480 | 7.4-11.5 | 0.17-4.5 | 2,120-3,510 | 13% (140) | + | ++++ | + | + | − | Mild anemia and thrombocytopenia, no therapy |
| 4 | M | 4 yr | 14 yr | 5-924 | 5.5-14.5 | 0.38-7.8 | 345-640 | 5% (100) | +++ | ++ | + | − | − | Severe neutropenia and thrombocytopenia, in therapy with PDN 1 mg/kg/d |
| 5 | M | 3 yr | 8 yr | 8-167 | 11.6-14.1 | 1.0-6.8 | 550-922 | 9% (150) | +++ | ++ | + | − | − | In remission, no therapy |
| 6 | M | 4 mo | 3 yr | 17-267 | 7.3-12.7 | 0.7-5.6 | 408-760 | 4% (120) | + | +++ | + | − | − | In remission, no therapy |
| 7 | M | 2 yr | 3 yr | 45-168 | 9.7-11.8 | 1.6-4.7 | 670-795 | 2% (53) | + | +++ | + | − | − | Mild thrombocytopenia, no therapy |
Abbreviations: Hb, hemoglobin; ANC, absolute neutrophil count; LAC, lupus anticoagulant (anticardiolipin antibodies); ANA, antinuclear antibodies; a-DNA, anti-DNA antibodies; PDN, prednisone.
Range observed since onset.
CD4 CD8 double negative T cells expressed as percentage of total TCRαβ+ lymphocytes and, in brackets, as absolute counts/μL of PB. DN T cells (mean ± SD) were 7 ± 4% (165 ± 118/μL) in patients with chronic ITP, 7 ± 5% (170 ± 25/μL) in age-matched normal controls, 6 ± 4% (113 ± 33/μL) in patients with ALD, and 22 ± 2% (452 ± 28/μL) in three patients with ALPs.
Lymphoadenopathy: +, 1-2 cm nodes; ++, 2-4 cm nodes; +++, one or more nodes >4 cm.
ρ Splenomegaly: +, 1-2 cm; ++, 3-4 cm; +++, 5-6 cm; ++++, >6 cm below costal margin.