Table 5 Initial and follow-up diagnostics in common variable immunodeficiency

Hematopoietic system

Blood formula and differential blood counts

(3-)6 months; more often in case of known

autoimmune cytopenia

Coombs test in any case of newly developing anemia

On demand

Bone marrow biopsy in case of suspicion of lymphoma or myelodysplasia

On demand at diagnosis or during follow-up

Immune system

IgG, IgA, IgM (aiming at trough levels >7g/L)

In intravenous immunoglobulin-substituted patients

Monthly trough levels

In subcutaneous immunoglobulin-substituted patients

1-3 months

Immunofixation in serum and urine, ß2-microglobulin

At diagnosis

CD3, CD4, CD8, CD19, CD56 lymphocyte subsets

Initially and repeatedly in case of suspected combined immunodeficiency

Vaccination responses to tetanus, diphtheria, hepatitis, pneumococcal polysaccharides

At diagnosis

Classification (detailed T/B-cell evaluation)

At diagnosis

Microbiology (direct culture of pathogens, PCR, antigen ELISA)

Purulent sputum: determine colonizing pathogens and resistance pattern to antibiotics

On demand

In case of chronic bronchiectasis, control of colonising pathogens (Pseudomonas spp., Haemophilius influenzae, Streptococcus pneumoniae, Staphylococcus aureus, Candida spp. and others) with sensitivity to antibiotics

6-12 months and on demand

Lungs

Spirometry, CO diffusion test, blood gases

12 months

Chest X-ray

(12-)24 months

HR-CT in case of proven GILD

24 months and on demand

Bronchoscopy + BAL in case of suspicion of GILD

At diagnosis; on demand

Lymphoproliferation

Abdomen sonography CT-Abdomen/MRT

12 months

Lymph node biopsy

On demand; in case of suspected lymphoma

Gastrointestinal tract

Oesophogastroscopy

In case of clinical symptoms and every

24 months in case of increased risk for developing intestinal malignancy

Colonoscopy

On demand

Central nervous system

MRT, liquor analysis in case of neurological symptoms (exclusion of enteroviral infection)

On demand