Characteristics of 10 asplenic patients
. | . | . | . | . | % of B cells . | . | Mutation frequency, % . | . | ||
|---|---|---|---|---|---|---|---|---|---|---|
| Patient, age . | Diagnosis . | Ultrasound and/or computerized tomography . | Howell-Jolly bodies . | Severe infections . | IgD+CD27+ . | IgD-CD27+ . | IgD+CD27+ . | IgD-CD27+ . | ||
| AS1 | ||||||||||
| 4 mo | Asplenia syndrome‡ | No spleen | + | None | 5 | 2 | ND | ND | ||
| 14 mo | 7 | 5 | 3.2 | 2.5 | ||||||
| AS2, 18 mo | Congenital asplenia | No spleen | + | Purpura fulminans¶ | 7 | 4.5 | 3.2 | 4 | ||
| AS3 | ||||||||||
| 23 mo | Functional asplenia | Small size§ | + | Meningitis, purpura fulminans, septicemia (S. pneumoniae) | 4 | 6 | 2.2 | 4 | ||
| 30 mo | 8 | 8.5 | ND | ND | ||||||
| AS4, 35 mo | Congenital asplenia | No spleen | + | Purpura fulminans¶ | 13 | 17 | ND | ND | ||
| AS5, 50 mo* | Congenital asplenia | No spleen | + | None | 3 | 8 | 3.7 | 4.7 | ||
| AS6, 5 y† | Congenital asplenia | No spleen | + | None | 9 | 11 | ND | ND | ||
| AS7, 18 y | Congenital asplenia | No spleen∥ | + | Septic shock with otitis¶ | 8 | 11 | ND | ND | ||
| AS8, 43 y | Congenital asplenia | No spleen | + | Septicemia (S. pneumoniae) | 5 | 14 | ND | ND | ||
| AS9, 52 y | Congenital asplenia | No spleen∥ | + | None | 3 | 5 | ND | ND | ||
| AS10, 71 y | Congenital asplenia | No spleen∥ | + | None | 11 | 3 | ND | ND | ||
. | . | . | . | . | % of B cells . | . | Mutation frequency, % . | . | ||
|---|---|---|---|---|---|---|---|---|---|---|
| Patient, age . | Diagnosis . | Ultrasound and/or computerized tomography . | Howell-Jolly bodies . | Severe infections . | IgD+CD27+ . | IgD-CD27+ . | IgD+CD27+ . | IgD-CD27+ . | ||
| AS1 | ||||||||||
| 4 mo | Asplenia syndrome‡ | No spleen | + | None | 5 | 2 | ND | ND | ||
| 14 mo | 7 | 5 | 3.2 | 2.5 | ||||||
| AS2, 18 mo | Congenital asplenia | No spleen | + | Purpura fulminans¶ | 7 | 4.5 | 3.2 | 4 | ||
| AS3 | ||||||||||
| 23 mo | Functional asplenia | Small size§ | + | Meningitis, purpura fulminans, septicemia (S. pneumoniae) | 4 | 6 | 2.2 | 4 | ||
| 30 mo | 8 | 8.5 | ND | ND | ||||||
| AS4, 35 mo | Congenital asplenia | No spleen | + | Purpura fulminans¶ | 13 | 17 | ND | ND | ||
| AS5, 50 mo* | Congenital asplenia | No spleen | + | None | 3 | 8 | 3.7 | 4.7 | ||
| AS6, 5 y† | Congenital asplenia | No spleen | + | None | 9 | 11 | ND | ND | ||
| AS7, 18 y | Congenital asplenia | No spleen∥ | + | Septic shock with otitis¶ | 8 | 11 | ND | ND | ||
| AS8, 43 y | Congenital asplenia | No spleen | + | Septicemia (S. pneumoniae) | 5 | 14 | ND | ND | ||
| AS9, 52 y | Congenital asplenia | No spleen∥ | + | None | 3 | 5 | ND | ND | ||
| AS10, 71 y | Congenital asplenia | No spleen∥ | + | None | 11 | 3 | ND | ND | ||
ND indicates not determined.
One asplenic brother died from invasive infection (Streptococcus spp)
One asplenic brother died from invasive infection (S. pneumoniae)
Or ivemark syndrome (visceral heterotaxia: right atrial isomerism, cardiac malformation and asplenia)
Not visualized by 99mTc sulfur colloid scintigraphy
Confirmed by 99mTc scintigraphy
Germ not identified