Table 3.

Characteristics of 10 asplenic patients

% of B cellsMutation frequency, %
Patient, ageDiagnosisUltrasound and/or computerized tomographyHowell-Jolly bodiesSevere infectionsIgD+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