Clinical Manifestations and Immunological Investigations
| Patient No. . | Sex/ Age (yrs) . | Age at Presentation (yrs) . | Lymph- adenopathy . | Spleno- megaly . | Splenec- tomy Age (yrs) . | Autoimmune Manifestations . | Serum Ig . | Lympho- cytes (/μL) . | TCR αβ/ CD4−CD8− (%) . | ||
|---|---|---|---|---|---|---|---|---|---|---|---|
| IgG . | IgA . | IgM . | |||||||||
| 1 | M/6 | 1.2 | +++ | +++ | − | Urticarial rash | ↗↗ | ↗↗ | N | 16,017 | 13 |
| 2 | F/15 | 1.5 | + | − | − | 0 | ↗↗ | N | ↘ | 1,800 | 6 |
| 3 | M/6 | 4 | − | +++ | − | 0 | ↗↗ | ↗ | ↗ | 1,600 | 6 |
| 4 | F/18 | 0.1 | ++ | ++ | − | Bilateral uveitis | ↗ | N | N | 1,600 | 23 |
| Iridocyclitis | |||||||||||
| Hemolytic anemia* | |||||||||||
| 5 | F/14 | 0.2 | ++ | ++† | + (2) | 0 | ↘ | ↘ | ↘ | 21,000 | 63 |
| 6 | F/15 | 4 | + | ++ | − | Anterior uveitis vasculitis | ↗↗ | ↗ | ↘ | 1,600 | 21 |
| Glomerulopathy | |||||||||||
| Thrombocytopenia* | |||||||||||
| 7 | M/12 | 3 | ++ | +++† | + (5) | Hemolytic anemia | ↗↗ | ↗↗ | N | 4,900 | 5 |
| 8 | M/24 | 6 | ++ | +++† | + (12) | Arthralgia | ↗↗ → N | ↗↗ → N | N | 2,728 | 3 |
| Urticarial rash | |||||||||||
| 9 | M/35 | 2 | ++ | +++† | + (16) | 0 | ↗↗ → N | ↗↗ | ↘ | 5,278 | 27 |
| 10a | M/11 | 3 | − | + | − | Skin vasculitis | ↗↗ | ↗↗ | ↘ | 2,420 | 10 |
| Guillain-Barre syndrome | |||||||||||
| 10b | M/12 | 4 | ++ | + | − | Skin vasculitis | ↗↗ | ↗ | ↘↘ | 1,540 | 5 |
| 11 | M/20 | 7 | +++ | +++ | + (11) | Hemolytic anemia* | ↗ | ↗ | N | 5,480 | 7 |
| Thrombocytopenia* | |||||||||||
| Neutropenia* | |||||||||||
| Arthritis | |||||||||||
| 12a | F/9 | neonatal | − | + | − | 0 | N | N | N | 1,200 | 3 |
| 12b | M/25 | 12 | + | ++† | + (14) | Thrombocytopenia* | ↗ | ↗↗ | ↘ | 5,000 | 1 |
| 13 | F/0.6 | neonatal | + | +++† | + (0.6) | 0 | ↗↗ | N | N | 19,650 | 65 |
| 14 | M/14 | neonatal | ++ | +++† | − | 0 | ↗↗ | ↗ | ↘ | 1,300 | 21 |
| Patient No. . | Sex/ Age (yrs) . | Age at Presentation (yrs) . | Lymph- adenopathy . | Spleno- megaly . | Splenec- tomy Age (yrs) . | Autoimmune Manifestations . | Serum Ig . | Lympho- cytes (/μL) . | TCR αβ/ CD4−CD8− (%) . | ||
|---|---|---|---|---|---|---|---|---|---|---|---|
| IgG . | IgA . | IgM . | |||||||||
| 1 | M/6 | 1.2 | +++ | +++ | − | Urticarial rash | ↗↗ | ↗↗ | N | 16,017 | 13 |
| 2 | F/15 | 1.5 | + | − | − | 0 | ↗↗ | N | ↘ | 1,800 | 6 |
| 3 | M/6 | 4 | − | +++ | − | 0 | ↗↗ | ↗ | ↗ | 1,600 | 6 |
| 4 | F/18 | 0.1 | ++ | ++ | − | Bilateral uveitis | ↗ | N | N | 1,600 | 23 |
| Iridocyclitis | |||||||||||
| Hemolytic anemia* | |||||||||||
| 5 | F/14 | 0.2 | ++ | ++† | + (2) | 0 | ↘ | ↘ | ↘ | 21,000 | 63 |
| 6 | F/15 | 4 | + | ++ | − | Anterior uveitis vasculitis | ↗↗ | ↗ | ↘ | 1,600 | 21 |
| Glomerulopathy | |||||||||||
| Thrombocytopenia* | |||||||||||
| 7 | M/12 | 3 | ++ | +++† | + (5) | Hemolytic anemia | ↗↗ | ↗↗ | N | 4,900 | 5 |
| 8 | M/24 | 6 | ++ | +++† | + (12) | Arthralgia | ↗↗ → N | ↗↗ → N | N | 2,728 | 3 |
| Urticarial rash | |||||||||||
| 9 | M/35 | 2 | ++ | +++† | + (16) | 0 | ↗↗ → N | ↗↗ | ↘ | 5,278 | 27 |
| 10a | M/11 | 3 | − | + | − | Skin vasculitis | ↗↗ | ↗↗ | ↘ | 2,420 | 10 |
| Guillain-Barre syndrome | |||||||||||
| 10b | M/12 | 4 | ++ | + | − | Skin vasculitis | ↗↗ | ↗ | ↘↘ | 1,540 | 5 |
| 11 | M/20 | 7 | +++ | +++ | + (11) | Hemolytic anemia* | ↗ | ↗ | N | 5,480 | 7 |
| Thrombocytopenia* | |||||||||||
| Neutropenia* | |||||||||||
| Arthritis | |||||||||||
| 12a | F/9 | neonatal | − | + | − | 0 | N | N | N | 1,200 | 3 |
| 12b | M/25 | 12 | + | ++† | + (14) | Thrombocytopenia* | ↗ | ↗↗ | ↘ | 5,000 | 1 |
| 13 | F/0.6 | neonatal | + | +++† | + (0.6) | 0 | ↗↗ | N | N | 19,650 | 65 |
| 14 | M/14 | neonatal | ++ | +++† | − | 0 | ↗↗ | ↗ | ↘ | 1,300 | 21 |
Lymphadenopathy: +, multiple small nodes <2 cm; ++, multiple nodes >2 cm <5 cm; +++, multiple and large nodes >5 cm. Splenomegaly: +, above umbilicus; ++, below umbilicus; +++, palpable in iliac fossa. Ig serum level: N, in normal range; ↗, >+2 SD <+4 SD; ↗↗, >+4 SD; ↘, <−2 SD.
All had autoantibodies.
Size before splenectomy.