Question

Jenny, a 6-year-old child who has been raised in a germ-free environment from birth, is a victim of one of the most severe examples of an abnormal immune system. Jenny also suffers from cancer caused by the Epstein Barr virus. Relative to this case: (a) What is the usual fate of children with Jenny's condition and similar circumstances if no treatment is attempted? (b) Why is Jenny's brother chose as the hematopoietic stem cell donor? (c) Why is her physician planning to use the umbilical cord blood as a source of stem cells for transplant if her brother's stem cells fail (what are the hoped for results)? (d) attempt to explain Jenny's cancer. (e) What similarities and dissimilarities exists between Jenny's illness and aids?

Answer 1

Children with SCID usually do not survive without treatment. Jenny's brother is chosen as the stem cell donor because of the genetic match. Umbilical cord blood is planned as an alternative if her brother's stem cells fail. Jenny's cancer is caused by the Epstein Barr virus. SCID and AIDS both weaken the immune system, but they have different causes and modes of transmission.

Step-by-step explanation:

(a) Without treatment, babies with Severe Combined Immunodeficiency (SCID) typically do not survive infancy. A bone marrow transplant can correct the defects in lymphocyte development that lead to SCID, but it carries risks. In the case of David Vetter, known as the "boy in the bubble," he received a bone marrow transplant from his sister but died from infection and Burkitt lymphoma due to a latent Epstein-Barr virus infection in the donor's bone marrow.

(b) Jenny's brother was chosen as the hematopoietic stem cell donor because he is a close genetic match, which increases the likelihood of a successful transplant.

(c) If her brother's stem cells fail, umbilical cord blood is planned as an alternative source of stem cells for transplantation. The hope is that these stem cells can successfully repopulate Jenny's bone marrow and reconstitute her immune system.

(d) Jenny's cancer is caused by the Epstein Barr virus, which is known to cause lymphomas like Burkitt lymphoma in individuals with compromised immune systems.

(e) Jenny's illness, SCID, is an example of primary immunodeficiency caused by a genetic disease. On the other hand, AIDS is a secondary immunodeficiency caused by the human immunodeficiency virus (HIV). Both conditions result in weakened immune systems, but they have different causes and modes of transmission.