[Agammaglobulinemia with the absence of circulating B-lymphocytes. 9 cases].
OBJECTIVE
Agammaglobulinemia with absence of circulating B lymphocytes is a rare genetically transmitted immunodeficiency that appears in early childhood and affect mainly boys. The clinical manifestations of the disease are rather heterogeneous.


METHODS
Nine patients (7 boys and 2 girls) were diagnosed as suffering from agammaglobulinemia with absence of circulating B lymphocytes, over a period of 6 years. Quantitation of immunoglobulins and search for circulating B lymphocytes were respectively performed by the Mancini method and immunofluorescence using T specific (anti-CD3, anti-CD4 and anti-CD8) and B (anti-CD19) monoclonal antibody.


RESULTS
The disease started to manifest clinically at the mean age of 8.7 months (4-16 months). The mean age at diagnosis is 4 years (1-11 years). The clinical manifestations were essentially recurrent infections of the lung and the gastrointestinal tract. However, bacterial meningitidis was observed in 3 patients. Severe complications such as an echovirus 27 meningoencephalitis and a chronic active hepatitis (1 patient) and a pericarditis (1 patient) were observed. All of our patients lacked circulating B lymphocytes and had low or null immunoglobulin levels. Five patients were treated by intravenous immunoglobulin (Ig) and 3 were treated by intramuscular immunoglobulin with a residual IgG level respectively of 5.5 g/l and 3.3 g/l.


CONCLUSIONS
Recurrent infections are the principal manifestation of the agammaglobulinemia, early Ig treatment is the only therapy allowing improved.
Centre National de Greffe de Moelle osseuse, Tunis, Tunisie.
M
M
Bejaoui
M R
MR
Barbouche
F
F
Mellouli
N
N
Tirellil
K
K
Dellagi
D000293:Adolescent; D000361:Agammaglobulinemia / Q000235:genetics / Q000276:immunology* / Q000628:therapy; D000367:Age Factors; D000911:Antibodies, Monoclonal; D018941:Antigens, CD19 / Q000032:analysis; D001402:B-Lymphocytes / Q000276:immunology*; D017252:CD3 Complex / Q000032:analysis; D015704:CD4 Antigens / Q000032:analysis; D016827:CD8 Antigens / Q000032:analysis; D002648:Child; D004457:Echovirus Infections; D005260:Female; D006801:Humans; D007116:Immunization, Passive; D007070:Immunoglobulin A / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D007075:Immunoglobulin M / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D007273:Injections, Intramuscular; D008231:Lymphopenia / Q000235:genetics / Q000276:immunology* / Q000628:therapy; D008297:Male; D016920:Meningitis, Bacterial / Q000209:etiology; D008590:Meningoencephalitis / Q000821:virology; D009894:Opportunistic Infections / Q000209:etiology; D012189:Retrospective Studies
D000911:Antibodies, Monoclonal; D018941:Antigens, CD19; D017252:CD3 Complex; D015704:CD4 Antigens; D016827:CD8 Antigens; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous
Agammaglobulinémie avec absence de lymphocytes B circulants. 9 observations.