Scuola di Specializzazione in Allergologia ed Immunologia Clinica (D.I. 68/2015)

Scuola di Specializzazione in Allergologia ed Immunologia Clinica (D.I. 68/2015)

Primary immunodeficiency disorders - DIDATTICA FRONTALE (2022/2023)

Course code

4S007198

Credits

2

Academic sector

MED/09 - INTERNAL MEDICINE

Language of instruction

Italian

Site

VERONA

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Syllabus

DEFINITION of Primary humoral immunodeficiencies
PRESENTATION OF HUMORAL IMMUNODEFICIENCY
The presence of an underlying primary humoral immunodeficiency should be suspected in a patient with typical sites of infections and types of infectious organisms isolated. Antibodies play an essential role in protection of the sinopulmonary tract and mucosal surfaces. Thus, affected persons commonly develop recurrent otitis media, sinusitis, and pneumonia. Recurrent pneumonias can lead to bronchiectasis, which serves as a negative factor for long-term morbidity and mortality
ASSESSMENT OF HUMORAL IMMUNODEFICIENCY
●Measurement of serum levels of IgG, immunoglobulin A (IgA), and immunoglobulin M (IgM)
●Assessment of functional antibody production
●Immunophenotyping of B cells by flow cytometry
●Genetic testing
CATEGORIES OF PRIMARY HUMORAL IMMUNODEFICIENCIES
-Agammaglobulinemia (severe reduction in all serum immunoglobulin isotypes with profoundly decreased or absent B cells)
-CVID phenotype (severe reduction in at least two serum immunoglobulin isotypes with normal or low number of B cells)
-Hyper IgM (severe reduction in serum IgG and IgA with normal/elevated IgM and normal numbers of B cells)
-Isotype, light chain, or functional deficiencies with generally normal numbers of B cells
DIFFERENTIAL DIAGNOSIS
TREATMENT

Assessment methods and criteria

Evaluation with oral exam of the learning of the exam program but in particular of the ability to apply this knowledge in the context of clinical activity directly with the patient

Reference books

See the teaching bibliography