Intraspinal tumor treated with autologous stem cell transplantation

[Leif]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17130114&query_hl=2&itool=pubmed_docsum


Primary intraspinal primitive neuroectodermal tumor treated with autologous stem cell transplantation: case report and review of the literature.

2007 Jan-Feb

Nutman A (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&itool=pubmed_AbstractPlus&term=%22Nutman+A%22%5BAuthor%5D), Postovsky S (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&itool=pubmed_AbstractPlus&term=%22Postovsky+S%22%5BAuthor%5D), Zaidman I (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&itool=pubmed_AbstractPlus&term=%22Zaidman+I%22%5BAuthor%5D), Elhasid R (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&itool=pubmed_AbstractPlus&term=%22Elhasid+R%22%5BAuthor%5D), Vlodavsky E (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&itool=pubmed_AbstractPlus&term=%22Vlodavsky+E%22%5BAuthor%5D), Kreiss Y (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&itool=pubmed_AbstractPlus&term=%22Kreiss+Y%22%5BAuthor%5D), Ben Arush MW (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Search&itool=pubmed_AbstractPlus&term=%22Ben+Arush+MW%22%5BAuthor%5D).

Israel Defense Forces, Medical Corps, Haifa, Israel.

The authors describe the case of a 19-year-old female patient with a primary primitive neuroectodermal tumor (PNET) of the thoraco-lumbar spinal cord, who presented with acute urinary retention and back pain for 2 months. Magnetic resonance imaging revealed an intradural extramedullary tumor, 6.5 cm long, in the region of the conus medullaris. Histological examination disclosed a small round cell tumor with immunohistochemical characteristics of a peripheral PNET. Metastatic workup showed no evidence of an intracranial tumor or metastases outside the neuroaxis. The patient received multidisciplinary treatment, including surgical excision, irradiation of the entire cranio-spinal axis, and high-dose chemotherapy with autologous stem cell rescue. Presently, 24 months after diagnosis, the patient remains in complete remission.

PMID: 17130114 [PubMed - in process]

[Wise Young]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17130114&query_hl=2&itool=pubmed_docsum

Leif, I should comment because the abstract used words that may be misleading to some people, concerning "autologous stem cell rescue". A PNET (primitive neuroectodermal tumor) is a kind of stem cell tumor, not unlike a teratoma, where the tumor shows markers of different kinds of cells. This tumor was outside of the spinal cord (extramedullary) but inside the dura (intradural). Because this kind of tumor may grow and metastasize, they decided to hit it hard with high dose chemotherapy. Most chemotherapy are devastating to bone marrow stem cells (and other stem cells of the body). So, what they did with this patient was that they removed some bone marrow and stored it while the patient received the high-dose chemotherapy. When the chemotherapy was over, they transplanted the bone marrow back. The bone marrow graft worked and the patient is now symptom free.

Autologous regrafting of bone marrow stem cells to people who are getting whole body radiation or chemotherapy for tumors is becoming very common. First practiced in children who were being treated for leukemia and lymphoma, this restores the bone marrow and stem cell populations to allow recovery of the immune system. The Department of Defense and Department of Homeland Security is very interested in this because they are very concerned about the consequences of a "dirty" bomb that might expose a large population of people to radiation. The primary effect of radiation is to kill the stem cells in the gut and bone marrow. People die a horrible death as their guts don't repair and their immune systems shut down. There is strong interest in the transfusion of multiple units of umbilical cord blood. For reasons that are still not well understood, umbilical cord blood don't have to matched perfectly in order to engraft in people who have immune compromise. For example, 4/6 HLA antigen match is usually sufficient for 80% of umbilical cord blood units to engraft while bone marrow from unrelated donors usually require 6/6 HLA match to engraft. Because there is no time for careful matching and the person is immune-compromised anyway (therefore reducing the chances of rejection), giving multiple units increases the likelihood that one or more of the units will engraft. Of course, autologous bone marrow cells would be a perfect match and should not be rejected.

A similar approach has been considered for people with auto-immune disorders such as chronic progressive multiple sclerosis. Such patients are producing antibodies or cells that are attacking their own myelin. By destroying the bone marrow with chemotherapy or whole body radiation, the auto-immune cells can be eliminated and then replaced by bone marrow or umbilical cord blood cells. In general, heterologous bone marrow (from another person) not only has to be carefully matched in order to prevent rejection, the transplanted bone marrow cells may regard the body they have been transplanted to as "foreign" and will attack the body, a phenomenon called "graft versus host" disease. Umbilical cord blood cells, perhaps because they are more immature, have a much lower incidence of graft-versus-host-disease. However, because umbilical cord blood have variable amounts of stem cells, they may not restore bone marrow function in adults. Umbilical cord blood has been much more successfully used in infants and children because of their smaller body size. However, one way around this is the administration of multiple units of umbilical cord blood.

Based on the above, Congress recently passed legislation to increase the number of umbilical cord blood units available for public transplantation in the United States, appropriating some $80 million to companies to collect umbilical cord blood and increase the number of banked units to over 100,000 in the United States. Please note, however, that this may not be enough. Several recent studies show that umbilical cord blood grafts can cure 90% or more of kids of thalassemia major (a disease that is similar to sickle cell anemia). There are millions of people with thalassemia major, particularly in malaria endemic countries such as Pakistan, Thailand, the Mideast, Indonesia, and southern China. There may be a run on the units of available umbilical cord blood. Imagine what would happen if a clinical trial were to show that chemotherapy and umbilical cord blood transplants were to stop multiple sclerosis with relatively low risk. In the United States alone, there may be as many as 400,000 people with serious multiple sclerosis that is causing loss of function. They would have to collect a lot of umbilical cord blood units to meet the demand.

Wise.

[Leif]

Thanks for clarifying. Also interesting to hear about MS, -and kids whit thalassemia major. We’ve had a case here now for some time debated concerning a kid whit thalassemia major, the reason is that it has been probated here for parents to create donor siblings for cases like this to use the siblings cells, those parents had to go to the US to find the eggs which could be used (sorted out), now we will have a change here as well in the legislations but the case with the thalassemia major kid has been discussed a lot, and you are right he also was an immigrant from Pakistan.

[Sue Pendleton]

Is there anywhere in the US currently doing the autogolous, sibling matched or umbilical cord renewal after radiation in chronic progressive (primary progressive) MS? If so is it as a clinical trial or will it be considered experimental by insurance companies and Medicaid/Medicare? I saw an article where this was done in Canada but so far if it is going on here I've missed it. Thanks