0867

THE EFFICACY OF IMMUNGLOBULIN REPLACEMENT TREATMENT IN THE LONG-TERM FOLLOW-UP OF THE B CELL DEFICIENCIES (XLA, HIM, CVID)

Benan Bayrakci, Flgen Ersoy, Vzden Sanal, Ihan Tezcan

Hacettepe University, Ankara, Turkey

Immunoglobulin replacement therapy is essential for the B cell deficiencies; X-linked agamaglobulinemia (XLA), hyper Ig M syndrome (HIM) and common variable immunodeficiency disease (CVID). As the immunoglobulin replacement therapy is expensive, the optimal dose, time period and serum Ig G levels for effective therapy should be well defined. For this purpose, the data of 46 patients (19 XLA, 7 HIM and 20 CVID) who received immunoglobulin replacement therapy regularly between 1984 and 2000, in Hacettepe University ]hsan Dopramac} Children's Hospital Immunology Unit, were analyzed. The incidence of infections and hospitalization rates were compared, regarding with immunoglobulin doses used and serum Ig G levels reached. By the mean dose of 350 mg/kg immunoglobulin monthly, and the mean serum Ig G level of 480 mg/dl maintained, the annual incidence of infections fell down from 12,4 to 3,2 (similar to the normal population) and annual hospitalization rates per patient fell down from 1,16 to 0,16 per year. Overall, serum Ig G levels of 300-500 mg/dl were found to be satisfactory, increasing the level over 500 mg/dl neither improved the prevention of pneumonia nor decreased the need for hospital admission. Only in CVID group serum Ig G levels above 500 mg/dl were found to be more efficient to control upper respiratory tract infections. Generally, the replacement interval of 4 weeks was found to be adequate, except for XLA patients. All the patients, except the ones accompanied by another illness, grew normally. Although the patients showed parallel development pattern to the general population only five of them were below the normal ranges.