| Summary: | Cases of CD 19 deficiency from South
East Asia has rarely been reported. A, 1l yr Malay boy
was referred with recurrent infections with 2 episodes
of'chickenpox at age 2 years. He had repeated episodes
of pneumoniae and bronchiectasis by age 6 years.
Physical examination revealed gross clubbing with
crepitations at both lung bases. IV lg administration
was instituted with clinical improvementlaboratory
data: Serum Ig (g/l )IgG 8.37[n 4.95- 16.56 ], IgA
0.92[n 0.30-2.35 7, lgM 0.21 [0.32-1.4}]Lymphocyte
subset (age 1l) CD19 Q o
, CD20 11.26% (12-22)CD3
83.64% (n 66-76) ,CD4 36.25 ( 33-41),CD8 41.55 (27-
35), CDl6+56 I1.6 %. (9-16),Specific antibody
response to polysaccharide antigen was impaired ;
whileNBT , lymphocyte proliferation to (PHA ) were
normal, Btk protein 18% (control 87.8 %). Our finding
conforms with a diagnosis of CD19 deficiency with
selective IgM deficiency and partial defective Btk
protein expression.
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