Table 1.

The diagnostic workup and follow-up are summarized

TimePlanned analyses
At clinical suspicion Detailed investigation of the infections 
Physical examination 
Laboratory tests: 
 Full blood count 
 Serum immunoglobulins (IgG, IgA, and IgM) 
 Tetanus antitoxoid antibodies 
 Lymphocyte subset absolute counts: CD3, CD4, CD8, CD19, and CD16 
 Cultures of sputum or nose-pharyngeal suction secretion 
 Bone marrow aspirate 
At diagnosis Laboratory tests: 
 Full blood count 
 Serum immunoglobulins (IgG, IgA, and IgM) 
 Extended T- and B-lymphocyte subsets (absolute counts): CD3; CD4; CD8; CD19; CD16; naïve and memory T cells; naïve, memory, and switched memory B cells 
 Response to booster vaccine if antitoxoid antibodies unprotective 
 Full blood count during acute infection to confirm normalization of the neutrophil count 
If suspected skin/anogenital lesions, consider a dermatologist evaluation and viral DNA analysis. 
If history of recurrent pulmonary infections, consider a pulmonologist evaluation and chest computed tomography (CT) scan. 
Every 6 mo Physical examination 
Consider a dermatologist and/or pulmonologist evaluation 
Pulmonary functional tests 
Laboratory tests: 
 Full blood count* 
 Serum immunoglobulins (IgG, IgA, and IgM) 
 Lymphocyte subsets (CD3, CD4, CD8, and CD19) 
 Culture of sputum or nose-pharyngeal suction secretion 
Chest CT scan (on the basis of clinical manifestations) 
TimePlanned analyses
At clinical suspicion Detailed investigation of the infections 
Physical examination 
Laboratory tests: 
 Full blood count 
 Serum immunoglobulins (IgG, IgA, and IgM) 
 Tetanus antitoxoid antibodies 
 Lymphocyte subset absolute counts: CD3, CD4, CD8, CD19, and CD16 
 Cultures of sputum or nose-pharyngeal suction secretion 
 Bone marrow aspirate 
At diagnosis Laboratory tests: 
 Full blood count 
 Serum immunoglobulins (IgG, IgA, and IgM) 
 Extended T- and B-lymphocyte subsets (absolute counts): CD3; CD4; CD8; CD19; CD16; naïve and memory T cells; naïve, memory, and switched memory B cells 
 Response to booster vaccine if antitoxoid antibodies unprotective 
 Full blood count during acute infection to confirm normalization of the neutrophil count 
If suspected skin/anogenital lesions, consider a dermatologist evaluation and viral DNA analysis. 
If history of recurrent pulmonary infections, consider a pulmonologist evaluation and chest computed tomography (CT) scan. 
Every 6 mo Physical examination 
Consider a dermatologist and/or pulmonologist evaluation 
Pulmonary functional tests 
Laboratory tests: 
 Full blood count* 
 Serum immunoglobulins (IgG, IgA, and IgM) 
 Lymphocyte subsets (CD3, CD4, CD8, and CD19) 
 Culture of sputum or nose-pharyngeal suction secretion 
Chest CT scan (on the basis of clinical manifestations) 
*

Close monitoring of neutrophil count in patients on G-CSF therapy.

Regular monitoring of serum immunoglobulin levels in case the treatment reaches optimal IgG serum levels (at least above 500 mg/dL).

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