Page 1164 - Clinical Immunology_ Principles and Practice ( PDFDrive )

CHaPtEr 82 Immune Reconstitution Therapy for Immunodeficiency 1128.e1

MUL t IPLE-CHOICE QUES t IONS

1. What distinguishes the kinetics of immune reconstitution C. Presence of active infections at the time of transplantation
following hematopoietic stem cell transplantation (HSCT) D. Age younger than 2 months of life
with unmanipulated bone marrow from a matched sibling E. Transplantation from a haploidentical donor
donor versus a T cell–depleted transplantation from a hap- 3. Use of reduced intensity conditioning regimens may reduce
loidentical donor in an infant with severe combined immu- drug-related toxicity but often results in mixed chimerism
nodeficiency (SCID)? after HSCT for primary immune deficiency (PID). In many
A. Transplantation from a matched sibling donor will provide cases, mixed chimerism is sufficient to control the disease
rapid engraftment of B lymphocytes. phenotype, but in some forms of PID, it is associated with
B. Transplantation from an human leukocyte antigen (HLA)– an increased risk of complications. Can you identify in which
matched donor allows rapid T-cell engraftment. of the following immunodeficiency this is the case?
C. Only transplantation from a matched sibling donor permits A. Familial hemophagocytic lymphohistiocytosis (FHL)
durable T-cell reconstitution. B. Chronic granulomatous disease (CGD)
D. Transplantation from an HLA-matched donor allows rapid C. Immune dysfunction/polyendocrinopathy/enteropathy/X-
multilineage hematopoietic engraftment. linked (IPEX) syndrome
E. There are no differences in the kinetics of immune D. Wiskott-Aldrich syndrome (WAS)
reconstitution.
E. Leukocyte adhesion defect type 1 (LAD-1)
2. Which of the following factors negatively affect outcome of
HSCT for SCID?
+
A. B SCID phenotype
B. History of previous infections