Dr. Edward Allan Sison, a former St. Baldrick’s Fellow, is a faculty member at Baylor College of Medicine and Texas Children’s Cancer Center. He’s researching ways to make chemotherapy more effective in children with high-risk leukemias. He explains APL leukemia symptoms, treatment options, and how your support is moving research forward to help kids with this disease.
What is acute promyelocytic leukemia?
Leukemia is a cancer of the white blood cells. Acute promyelocytic leukemia (APL) comes from a type of white blood cells called promyelocytes.
Normal promyelocytes will grow up into white blood cells that fight off infection. In APL, the promyelocytes forget that they are supposed to grow up, and instead multiply at a very fast rate.
What are the signs and symptoms of acute promyelocytic leukemia?
Patients with APL may have fatigue, weight loss, bone pain, headache, or fever. Patients may also bruise and bleed easily.
If leukemia cells find their way into the protective fluid that surrounds the brain, patients may have blurry vision, headaches, seizures, nausea, or a facial droop.
Physical examination may also reveal tiny red dots called petechiae, abdominal tenderness, or enlargement of the liver or spleen.
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How is acute promyelocytic leukemia diagnosed?
The complete blood cell count is a routine blood test that can be used to diagnose acute promyelocytic leukemia.
The count is performed by a machine that measures the amount of red blood cells, white blood cells, and platelets in the bloodstream. The machine can also analyze the types of white blood cells.
If the machine finds white blood cells that look abnormal, a technologist or doctor will review the blood under the microscope to determine if the cells look like APL cells.
Patients with APL may also have low hemoglobin (anemia) or low platelet counts on their complete blood cell count.
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The diagnosis of APL is then confirmed by taking a sample from the patient’s bone marrow. The bone marrow is the center cavity inside the bones and is the factory that produces all blood cells, including leukemia cells. A pathologist will perform several tests on the bone marrow sample to make the diagnosis of APL.
What treatment options are available to patients with acute promyelocytic leukemia?
Patients with APL can be treated with standard chemotherapy, as well as medications that are not considered to be traditional chemotherapy.
These non-chemotherapeutic medications are called all-trans retinoic acid and arsenic trioxide. All-trans retinoid acid and arsenic trioxide can both kill the leukemia cells and cause the leukemia cells to grow up into normal white blood cells.
Until recently, children with APL were treated with a combination of arsenic trioxide, all-trans retinoic acid, and chemotherapy.
How is research helping kids with acute promyelocytic leukemia?
A clinical trial in adults with APL changed the treatment plans for all patients with acute promyelocytic leukemia, including children.
This clinical trial compared all-trans retinoic acid plus arsenic trioxide to all-trans retinoic acid with chemotherapy.
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Compared to patients who received chemotherapy, patients who received all-trans retinoic acid and arsenic trioxide had higher cure rates and longer rates of survival.
Because of the results from this clinical trial, the Children’s Oncology Group, or COG, is conducting their own clinical trial for children with acute promyelocytic leukemia.
Through this clinical trial, the COG is hoping to demonstrate that the chemotherapy-free regimen of all-trans retinoic acid and arsenic trioxide will also result in higher cure rates in children with acute promyelocytic leukemia.
Because these children will not be receiving chemotherapy, the hope is that children treated in this clinical trial will not develop long-lasting side effects related to their treatment.
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