AGAMMAGLOBULINEMIA WITH ABSENCE OF CIRCUL ATING B-LYMPHOCYTES - A SERIES OF 9 CASES

OBJECTIVES: Agammaglobulinemia with absence of circulating B lymphocyt es is a rare genetically transmitted immunodeficiency that appears in early childhood and affect mainly boys. The clinical manifestations of the disease are rather heterogeneous, PATIENTS AND METHODS: Nine pati ents (7 boys and 2 girls) were diagnosed as suffering ring from agamma globulinemia 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 i mmunofluorescence using T specific (anti-CD3, anti-CD4 and anti-CD8) a nd B (anti-CD19) monoclonal antibody. RESULTS: The disease started to manifest clinically at the mean age of 8,7 months (4-16 months). The m ean age at diagnosis is 4 years(1-11 years). The clinical manifestatio ns were essentially recurrent infections of the lung and the gastroint estinal tract. However, bacterial meningitidis was observed in 3 patie nts. Severe complications such as an echovirus 27 meningoencephalitis and a chronic active hepatitis (1 patient) and a pericarditis (1 patie nt) were observed. All of our patients lacked circulating B lymphocyte s and had low or null immunoglobulin levels. Five patients were treate d by intravenous immunoglobulin (lg) and 3 were treated by intramuscul ar immunoglobulin with a residual IgC level respectively of 5,5g/l and 3,3 g/l. CONCLUSION: Recurrent infections are the principal manifesta tion of the agammaglobulinemia. early Ig treatment is the only therapy allowing improved.