RT Book, Section A1 Lichtman, Marshall A. A1 Kaushansky, Kenneth A1 Kipps, Thomas J. A1 Prchal, Josef T. A1 Levi, Marcel M. SR Print(0) ID 1126653074 T1 Primary Immunodeficiency Syndrome T2 Williams Manual of Hematology, 8e YR 2011 FD 2011 PB McGraw-Hill Education PP New York, NY SN 9780071622424 LK hemonc.mhmedical.com/content.aspx?aid=1126653074 RD 2024/11/02 AB Primary immune deficiency diseases (PIDDs) are characterized by increased susceptibility to infections and are determined by the failure of either the humoral or cellular arms of the immune system or both.The clinical features of PIDDs are listed in Table 51–1.— Characterized, principally by recurrent pyogenic bacterial infections, including sinusitis, furunculosis, and recurrent or chronic pneumonias that often terminate in bronchiectasis. These infections are initially responsive to antibiotics but soon relapse.Evaluation of serum immunoglobulin (Ig) levels and specific antibody responses in patients with recurring infections without an apparent predisposing cause should be made.— Baseline Ig levels are often low or virtually absent.— Antibody response to immunization is often inadequate.Abnormality of cellular immunity causes:— Susceptibility to viral, protozoal, and fungal infections.— Patients are often anergic. Rejection or clearance of allogeneic cells may be impaired.— There may be a secondary defect in humoral immunity because of T-cell dysfunction and loss of B-cell helper activity.Autoimmune diseases such as immune-mediated hemolytic anemia, thrombocytopenia, or rheumatoid arthritis-like conditions occur at a higher frequency in certain primary immunodeficiency states.The more severe primary immune deficiencies are usually present in infancy, although common variable immunodeficiency (CVID) often presents later in life.In Ig-deficient patients, treatment with intravenous immunoglobulin (IVIG) may decrease infectious events.The autosomal recessive syndromes are frequently the result of consanguineous marriages.