Acute erythroblastic leukemia, also called erythremic myelosis, DiGuglielmo syndrome, or erythroleukemia, results from uncontrolled proliferation of immature erythrocytes (red blood cells).
Description
Acute erythroblastic leukemia, a variant of acute myelocytic leukemia, originates in the blood and in the bone marrow. In this form of leukemia, a large number of abnormal, immature red blood cells are produced. The advanced phase is also called the blast crisis. At this stage, over 50% of the cells in the bone marrow are immature malignant cells (also called blast cells or promelocytes).
Demographics
There are no statistics available for this rare form of cancer.
Causes and symptoms
The causes of acute erythroblastic leukemia are largely unknown. However, acute erythroblastic leukemia constitutes 10-20% of leukemias secondary to radiation, alkylator therapy, or overexposure to benzene.
Patients with this type of leukemia have less than the normal amount of healthy red blood cells and platelets, which results in insufficient amounts of oxygen being carried through the body. This condition is called anemia, and causes patients to experience severe weakness and tiredness. Patients may have less than the normal number of white blood cells as well. Other symptoms include fever, chills, loss of appetite and weight, easy bleeding or bruising (due to lower than normal platelet levels), bone or joint pain, headaches, vomiting, and confusion. In addition, patients with leukemia may have hepatosplenomegaly, an enlargement of the liver and spleen. Enlargement of these organs is noticed as a fullness or swelling in the abdomen, and can be felt by a doctor during a physical examination. The occurrence of Sweet's syndrome, a rare skin disorder accompanied by fever, inflammation of the joints (arthritis), and the sudden onset of a rash, has also been associated with acute erythroblastic leukemia.
Diagnosis
Patients seeking treatment usually report a vague history of chronic general fatigue. Blood tests are used to establish the diagnosis. A sample of blood is examined
under a microscope to identify abnormal red cells— which are larger than healthy cells—and to count the number of mature cells and blasts present. Cancer red cell precursors predominate, myeloid blasts also are seen, and multinucleated red cell precursors are common. Bone marrow examinations are also performed, either by aspiration or biopsy to examine the cell types further.
Treatment
Treatment for acute erythroblastic leukemia depends on the features of the cancer cells present and on the extent of the disease, as well as on the age of the patient, his symptoms, and general health condition. This disease can have an indolent course and may only require observation in the early stages. The treatment strategy is based on chemotherapy and in some patients, bone marrow or cell transplantations are indicated as well. Chemotherapy is usually administered in combinations of two or more drugs. Post-remission therapy includes maintenance chemotherapy for most patients.
Clinical staging, treatments, and prognosis
Acute erythroblastic leukemia is a very aggressive form of leukemia and does not respond well to the types of therapy used for a related type of cancer known as acute myelocytic leukemia. However, recent advances in chemotherapy protocols and bone marrow transplantation techniques, either allogeneic or autologous, have been identified as a means to increase the cure rate. The patient's cancerous bone marrow is first purged using drugs and radiation therapy before being replaced by healthy bone marrow that is obtained from a suitable donor (allogeneic) or from the patient himself (autologous). In the case of an autologous transplant, the bone marrow is treated outside the
patient's body to remove the cancer cells before transplantation.
Coping with cancer treatment
Like all types of leukemias, patients with acute erythroblastic leukemia usually experience a number of
specific complications and side effects resulting from treatment, as well as emotional concerns. They require supportive care to cope with these issues and to maintain their comfort and quality of life during treatment. As with every serious disease, psychological stress is increased and it is important for patients to be able to discuss their needs and concerns about tests, treatments, hospital stays, and financial consequences of the illness. Family, friends, doctors, nurses, and other members of the health care team are the best sources of support, as well as social workers, counselors, and members of the clergy.
Clinical trials
In 2001, no clinical trials for this leukemia were registered with the National Cancer Institute.
Prevention
Since this form of cancer is very rare and its causes are largely unknown, no specific preventive measures can be recommended.
Special concerns
Patients diagnosed with acute erythroblastic leukemia require special support in that they must deal with having a rare form of cancer about which there is very little specific information available. This creates additional anxiety and special care must be taken to explain to the patient that an uncommon cancer is not an untreatable one.
Jandl, James H. Blood. Textbook of Hematology. Boston: Little, Brown, 1996.
PERIODICALS
Shichishima, T., "Minimally Differentiated Erythroleukemia:Recognition of Erythroid Precursors and Progenitors." Internal Medicine 39 (October 2000): 843-46.
Mazzella, F.M., et al. "The Acute Erythroleukemias." Clinical Laboratory Medicine 20 (March 2000): 119-37.
Novik, Y., et al. "Familial Erythroleukemia: A Distinct Clinical and Genetic Type of Familial Leukemias." Leuk. Lymphoma 30 (July 1998): 395-401.
ORGANIZATIONS
National Cancer Institute, Public Inquiries Office, Building 31, Room 10A31, 31 Center Drive, MSC 2580, Bethesda, MD 20892-2580. (301)435-2848. <http://www.nci.nih.gov>.
National Cancer Information Center. 1-800-ACS-2345. The Leukemia and Lymphoma Society of America. 1-800-955-4572. <http://www.leukemia-lymphoma.org/>.
OTHER
American Cancer Society's Consumer Guide to Cancer Drugs. Caregiving—A Step-by-Step Resource for Caring for the Person with Cancer at Home. Available from: American Cancer Society. (800) ACS-2345. <http://www.cancer.org.>.
Advanced Cancer: Living Day by Day. Chemotherapy and You: A Guide to Self-help During Treatment. Eating Hints for Cancer Patients. What You Need to Know About Leukemia. Available from: National Cancer Institute, National Institute of Health. (800) 4-CANCER. <http://www.nci.nih.gov>.
Monique Laberge, Ph.D.
Allogeneic bone marrow transplant
—A bone marrow transplant using bone marrow obtained from a genetically matched healthy donor, such as a sister or a brother.
Autologous bone marrow transplant
—A bone marrow transplant that uses the patient's own bone marrow.
Anemia
—A condition in which the number of red blood cells is below normal
Blast cells
—Immature cancer cells. Also called promelocytes.
Bone marrow aspiration
—Common technique used to obtain a bone marrow sample from a patient. A needle is inserted into a marrow-containing bone, such as the hip (iliac crest) or sternum (breast bone) and a small amount of liquid bone marrow is removed for examination.
Bone marrow biopsy
—Another common technique used to obtain a bone marrow sample from a patient. Like bone marrow aspiration, it is performed with a needle, but a larger one is used and a small piece of bone is removed as well as bone marrow.
Chemotherapy
—The treatment of disease by means of chemicals. In cancer, the chemicals selectively destroy cancerous tissue. When cancer remission occurs, a course of maintenance chemotherapy is often prescribed so as to prevent recurrence.
Erythrocyte
—Red blood cell.
Leukemia
—Cancer of the blood-forming tissues.
Myeloid blast cell
—Type of cancer cell originating in the bone marrow.
Platelet
—A type of blood cell responsible for blood coagulation and for the repair of damaged blood vessels.
