TY - CHAP M1 - Book, Section TI - Primary Immunodeficiency Syndrome A1 - Lichtman, Marshall A. A1 - Kaushansky, Kenneth A1 - Prchal, Josef T. A1 - Levi, Marcel M. A1 - Burns, Linda J. A1 - Armitage, James O. PY - 2017 T2 - Williams Manual of Hematology, 9e AB - Primary immune deficiency diseases (PIDDs) are characterized by increased susceptibility to infections and result from the failure of either the humoral or cellular arms of the immune system or both.The clinical features of PIDDs are listed in Table 50–1.— PIDDs are characterized 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 recur.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 may occur.— 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - hemonc.mhmedical.com/content.aspx?aid=1133364509 ER -