RT Book, Section
A1 Lichtman, Marshall A.
A1 Kaushansky, Kenneth
A1 Prchal, Josef T.
A1 Levi, Marcel M.
A1 Burns, Linda J.
A1 Armitage, James O.
SR Print(0)
ID 1133364509
T1 Primary Immunodeficiency Syndrome
T2 Williams Manual of Hematology, 9e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9781259642470
LK hemonc.mhmedical.com/content.aspx?aid=1133364509
RD 2024/04/20
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.