Question

Susan and Joe were worried. Their little boy, Daniel, had been having an awful lot of bacterial infections and he was barely a year old. It seemed that the antibiotics cleared up one bacterial respiratory infection only to have another follow shortly. The scary thing was that Daniel had just fought off a case of pneumonia caused by Pneumocystis carnii, a fungal infection that was usually found in people with HIV. Waiting for the test results of an HIV test for their little boy was one of the worst waits ever. Thank goodness it came back negative. However, it seemed that their troubles were just beginning. After this last lung infection, the fungal one, and a negative HIV test, their doctor had ordered a number of other blood tests, including a genetic test that Susan didn’t fully understand. Apparently the doctor was worried about Daniel’s immune system functions. Susan had also met with a genetic counselor who collected a family history of any immune disorders. The details were vague, but Susan’s mother, Helen, knew that one of her three brothers had died young from an unexplained lung infection. Unfortunately, Grandma Ruth had passed away a few years ago, leaving them with numerous unanswered questions. Susan and Joe had an appointment with their doctor that afternoon to go over the results. When they arrived Dr. Dresdner led them into an office where Ms. Henchey, the genetic counselor, waited. This can’t be good, thought Susan. The doctor began by explaining that they had analyzed Daniel’s blood and found that while he had normal levels of B cells and T cells, his antibody levels were anything but normal. The levels of IgG, IgA, and IgE were very low, almost undetectable, and Daniel had abnormally high levels of IgM and IgD. He went on to explain the nature of these different categories of immunoglobulins.

Dr. Dresdner explained that there was treatment for this disease. Daniel would need immunoglobulin infusions every three to four weeks in order to provide him with missing IgG and hopefully lower the abnormally high level of IgM in his bloodstream. Also, although current therapies could result in a happy, healthy adult, Daniel was at risk for an increased rate of liver disease. However, the doctor stated, there was a potential cure. A bone marrow transplant or cord blood stem cell transplantation, particularly from a matching HLA sibling, could provide a permanent cure. Someday, the doctor stated, gene therapy may be an option, but not at the present moment.

How can a bone marrow or cord blood stem cell transplant cure Daniel? Explain

Answer 1

1. The primary lymphoid organs in the body are thymus and bone marrow from where all the immunoglobulins arise. The B cells T cells arise from bone marrow fight against the antigens. A bone marrow transplantation can replace the stem cells of the bone marrow which will start to synthesize the immunoglobulins to fight against diseases that occur due to the lack of particular antigen. The cord blood is retrieved from the umbilical cord and placenta after baby's birth can able to synthesize all immunoglobulins. So the cord blood stem cell transplantation can generate the bone marrow which can produce all immunoglobulins in required amounts. This method of approach can permantly cure the immunoglobulins insufficiency and can control the over production of certain immunoglobulins.