The Hereditary Hematologic Malignancy Resource is our newest addition. It began in the fall of 1999. Since that time, we have registered more than 60 families into our database. Our goal is to find the cause(s) of hematologic cancers. We have formed consortiums with professionals in the field all over the world.
We are not in the envious position as with solid tumors where a litany of cancer causing germ-line mutations have been identified. Unfortunately, we have a lot to learn. Although we are low on the learning curve for a genetic knowledge about hematologic cancers, there is encouraging news. For example, we believe we are close to identifying susceptibility gene loci for chronic lymphocytic leukemia. At this point, however, there is nothing to be done prophylactically.
Another encouraging development involves the work of Brian J. Druker, M.D., and colleagues who are credited with basic and clinical research on imatinib, a selective inhibitor of the BCR-ABL tyrosine kinase, which produces high response rates in patients with chronic-phase chronic myeloid leukemia (CML).1, 2
Imatinibtm has been shown to induce high rates of cytogenetic and hematologic responses in patients with chronic-phase CML in whom previous interferon therapy had failed.
This BCR-ABL tyrosine kinase inhibitor, ST1571 (Imatinibtm or Glivectm), is well tolerated and also has substantial activity in the blast-crisis of CML. It also has activity in Philadelphia chromosome-positive acute lymphocytic leukemia (ALL). Thus, we see how knowledge of molecular genetics in this hematologic cancer has led to a highly-targeted therapeutic product that has shown substantial therapeutic efficacy.3
Hematologic malignancies account for almost 10% of all cancers and include many different diseases. The common denominator for all of them is that they are considered cancers of the hematologic and immune systems. The major ones include:
Acute or Chronic Myeloid Leukemia (AML or CML)
Myelodysplastic Syndromes and Myeloproliferative Diseases
Acute or Chronic Lymphocytic Leukemia (ALL or CLL)
There are also several hereditary disorders that predispose to hematologic malignancies:
Ataxia telangiectasia (Louis-Bar syndrome)
Fanconi’s aplastic anemia
We are deeply committed to studying all varieties of hereditary hematologic cancer. We want to hear from you, especially if your family has two or more blood relatives affected with a hematologic malignancy. We are also investigating the occurrence of these cancers with other kinds of cancer within the family. You can make a difference in helping fight this group of cancers.
The information you are asked to provide in all your contacts with the Department of Preventive Medicine at Creighton University Medical Center Hereditary Cancer Institute (CUMC-HCI) is personal and confidential. We will add this information to our computerized data files on the history of cancer in families, and use it in our studies of hereditary cancer. Computers holding family data are not connected to outside computers or cabled dial-in networks.
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