Genetic Abnormality May Increase Risk Of Blood Disorders

Source: ScienceDaily (Mar. 17, 2009)

Researchers have found for the first time that a tendency to develop some blood disorders may be inherited. There is a common common genetic sequence abnormality that enhances the likelihood of acquiring a mutation in a gene linked to certain blood diseases.


The investigators carried out a genome-wide study to identify inherited DNA sequence changes that frequently occur in patients with myeloproliferative neoplasms, in which several types of blood cells are excessively produced in the bone marrow. They found that an inherited alteration in the gene for JAK2 – (a protein with enzymatic activity that is linked to the abnormal production of blood cells) – is more common in patients with these disorders.

According to the authors, understanding the underlying inherited sequence partly explains the predisposition for acquiring mutations in certain disease-specific genes and may help explain why some individuals are at higher risk in developing the disease. This is only the beginning to understand how some people may develop these blood disorders and others not.

Childhood Blood Cancer Survival Rates Improve

HEIDELBERG, Germany, Sept. 10 -- Survival rates for the four major childhood blood cancers are continuing to rise, researchers here said.

The survival rate has improved over the last 15 years. Five- and 10-year survival rates for U.S. children with acute lymphoblastic leukemia, Hodgkin's lymphoma, and non-Hodgkin's lymphoma are approximately 90% and for acute nonlymphoblastic leukemia lags at less than 60%.


A period analysis of the years 2000 through 2004, for instance, would reflect survival of all patients during those five years, regardless of when they were diagnosed. In contrast, the usual method would look at all patients newly diagnosed during those years, a process that could not be completed until years later.

The SEER database included 3,622 children diagnosed with both forms of leukemia over the three time periods and 1,045 with the lymphomas.

Analysis showed that the five- and 10-year survival rates during 1990 through 1994 were:

80.2% and 73.4% for acute lymphoblastic leukemia
41.9% and 38.7% for acute nonlymphoblastic leukemia
76.6% and 73% for non-Hodgkin's lymphoma
96.1% and 94.4% for Hodgkin's lymphoma

The researchers also used a new modeling technique to project 10-year survival rates for children diagnosed in the period from 2005 through 2009 and suggested they would be 88% for acute lymphoblastic leukemia, 63.9% for acute non-lymphoblastic leukemia, 90.6% for non-Hodgkin's lymphoma, and 94.3% for Hodgkin's lymphoma.

The study is good news for parents, patients, and society as a whole, according to Alan Wayne, M.D., of the National Cancer Institute, and colleagues.

How 11-year-old Breanna Beat Cancer

"Growing pains" was the initial diagnosis Breanna Atwell's pediatrician gave for the 11-year-old's throbbing joints and bones, but instead it was leukemia. She was diagnosed with acute lymphocytic leukemia, a blood cancer.

Within hours, she was on chemotherapy. For eight months, she had chemo several times a week: pills, an IV drip, and injections into muscle tissue and spinal fluid.

Luckily, Breanna was a "rapid responder." Just two weeks into her chemotherapy, the leukemia cells had plummeted from 70 percent of her bone marrow cells to 3 percent, and by the fourth week, no cancerous cells could be found.

Breanna had to return regularly for more than two years of chemotherapy that dulled her memory and weakened her body. She had six transfusions because her platelet and red blood cell counts took a dive. She had blood clots and bloodstream infections.

Two years and three months of treatment finally came to a close last month with one last intravenous chemo, removal of the central line from Breanna's chest, and a ceremonious disposal of her leftover chemo pills. But Breanna will need to return to Children's periodically for at least 10 years, says Villavicencio: "From her growth and development to how she's doing in school and getting along with her family—we're interested in absolutely everything."

Support Groups and Counseling

Living with leukemia presents many new challenges for you and for your family and friends.

Many people feel anxious and depressed. Some people feel angry and resentful; others feel helpless and defeated.

For most people with leukemia, talking about their feelings and concerns can be therapeutic. Many people with leukemia are profoundly helped by talking to other people who have leukemia. My family and friends helped me during my ordeal. I also spoke to others who had leukemia. The hospital I stayed at also had additional support groups I could contact.

The American Cancer Society also has information about support groups all over the United States. For more information about support groups, contact the following agencies:

American Cancer Society: 800-ACS-2345

National Cancer Institute, Cancer Information Service: 800-4-CANCER (800-422-6237); TTY (for deaf and hard-of-hearing callers): 800-332-8615

Leukemia and Lymphoma Society: 800-955-4572

Cyclophosphamide May Increase Cancer Risk Among Patients with Rheumatoid Arthritis

A large study conducted in Canada reveals that patients who are treated for rheumatoid arthritis (RA) with the drug cyclophosphamide may have an increased risk for developing hematological cancers such as lymphoma.

There is no cure for RA, though proper treatment can control symptoms and allow patients to lead long, productive lives. There are several treatments for this disease.

Cyclophosphamide is an immunosuppressive drug commonly used to treat not only RA but also certain types of cancer.

Research has been done and in this study researchers recruited 23,810 patients with RA between 1980 and 2003. The focus of the study was to determine if anti-rheumatic drugs were associated with risk for cancers of the blood or lymph systems.

619 patients developed cancers of the blood or lymph systems.
346 patients developed lymphoma.
178 patients developed leukemia.
95 patients developed multiple myeloma.

These results indicate that although immunosuppressant drugs in general may increase the risk for cancers of the blood or lymph system, cyclophosphamide had the greatest risk for lymphoma.

New Therapy for Non-Hodgkin Lymphoma

There is a more effective method to evaluate response to radiation therapy, using the combined positron emission tomography (PET) and computed tomography (CT) imaging of lymphoma patients. It may help patients avoid unnecessary follow-up treatments.

The clinical study is the first to show the advantage of combined PET-CT imaging in evaluating radioimmunotherapy for patients with non-Hodgkin lymphoma, says Gary Ulaner, M.D., Ph.D., radiology resident and nuclear medicine fellow at the Keck School of Medicine of USC and the principal investigator of the study.

Radioimmunotherapy is a type of radiation treatment that targets cancer cells through antibodies. It is said it has less side effects.

PET is a type of nuclear medicine imaging that uses very small amounts of radioactive material to diagnose or treat disease and other abnormalities.

"Using this relatively novel hybrid imaging means that some patients will only require a one-day treatment of radioimmunotherapy instead of several rounds of chemotherapy," says Peter Conti, M.D, Ph.D., professor of radiology at the Keck School of Medicine. "We will have a more accurate image that will identify the patients who have a complete response to the treatment."

Conti is a national leader in treatment of non-Hodgkin lymphoma with radioimmunotherapy.

"We hope this research will result in PET-CT imaging becoming the standard for evaluating patients after treatment," he says. "Greater accuracy will improve how physicians care for lymphoma patients."

Types of Leukemia

Types of Leukemia

There are two main classifications of leukemia, acute leukemia and chronic leukemia. Within each of these categories, the cancerous cell type determines the type of either acute or chronic leukemia. Many people are not familiar with the different types of the disease.

Acute leukemia
Acute leukemia is an aggressive, life-threatening disease. Acute leukemia is usually defined by a blast (immature white blood cell) count of greater than 20 percent in the bone marrow.

Acute myelogenous leukemia (AML)

Acute myelogenous leukemia (AML) is when the myeloid cell becomes cancerous. There are several subtypes of AML that are very similar in terms of prognosis and treatment. The patient who has been diagnosed with AML is usually hospitalized right away in order to start therapy. The usual treatment is intensive induction chemotherapy.

Acute promyelocytic leukemia (APL)
Acute promyelocytic leukemia (APL) is a subtype of AML that differs slightly from the other subtypes of AML. APL is associated with bleeding and will respond better when a specific drug (all-trans-retinoic acid – ATRA) is used in combination with standard chemotherapy.

Acute lymphocytic leukemia (ALL)
Acute lymphocytic leukemia (ALL) is the most common leukemia in children but may also occur in adults. In ALL, the lymphoid cells become cancerous. Treatment for acute lymphoctic leukemia includes induction chemotherapy with the goal of achieving remission (the bone marrow biopsy shows no evidence of leukemia).

Chronic leukemia
Chronic leukemia has a slower onset and is not usually life-threatening at the time of diagnosis. Chronic leukemia has a longer natural history, meaning that if the disease were untreated, people would survive significantly longer than those with acute leukemia. There are significant differences in the approaches to the various types of chronic leukemia.

Chronic myelogenous leukemia (CML)
Chronic myelogenous leukemia affects the myeloid cells. The disease usually affects people in their 50s and 60s but may affect a person at any age. The diagnosis of CML can be confirmed in most cases by the presence of an abnormal gene or chromosome - the Philadelphia chromosome.

There are three phases of CML: the chronic phase, which may last years; the accelerated phase, which may last months; and blast crisis, which is treated more like acute myelogenous leukemia (AML).

Chronic lymphocytic leukemia (CLL)
Chronic lymphocytic leukemia generally occurs in older adults and progresses slowly. People generally have no or few symptoms and since CLL is not curable, it is usually treated conservatively. CLL is the one type of leukemia that has a formal staging system.

Hairy cell leukemia
Hairy cell leukemia is a rare type of chronic leukemia in which the leukemia cells appear to have hair when examined under the microscope. About 85 percent of people with hairy cell leukemia will respond very well to the chemotherapy. Many remissions will last longer than 10 years and treatment can be repeated.