From the Guidelines
Vision changes in acute promyelocytic leukemia (APL) are a significant concern and can occur due to the disease itself or its treatment, necessitating prompt recognition and management to prevent permanent vision loss. Vision changes in APL can be attributed to several factors, including retinal hemorrhages resulting from coagulopathy with low platelet counts and disseminated intravascular coagulation, as well as hyperviscosity from high white blood cell counts impairing retinal blood flow 1.
Key Considerations
- Retinal hemorrhages can lead to blurred vision, visual field defects, or even blindness
- High white blood cell counts can cause hyperviscosity, impairing retinal blood flow
- Treatment with all-trans retinoic acid (ATRA) can cause pseudotumor cerebri, associated with papilledema and visual disturbances in approximately 10-15% of patients
- Arsenic trioxide, another APL treatment, can occasionally cause optic neuropathy
Management
- If a patient on ATRA therapy reports headaches and visual changes, the medication should be temporarily discontinued and urgent ophthalmologic evaluation obtained
- Management may include acetazolamide 250-500 mg twice daily to reduce intracranial pressure, along with possible lumbar puncture for diagnostic and therapeutic purposes
- Regular ophthalmologic examinations are recommended during APL treatment, especially for patients on ATRA therapy, as suggested by the international expert panel recommendations for the diagnosis and management of acute myeloid leukemia in children and adolescents 1.
From the Research
Vision Changes in Acute Promyelocytic Leukemia
- Vision changes can be a presenting symptom of acute promyelocytic leukemia (APL), as seen in a study where patients presented with neuro-ophthalmologic signs such as severe hemorrhagic papilledema, hemorrhagic bilateral optic disc edema, and homonymous hemianopia 2.
- These vision changes can be related to complications such as dural venous sinus thrombosis or occipital lobe hemorrhage, highlighting the importance of prompt diagnosis and treatment of APL 2.
- Treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) can be effective in managing APL, but may also cause side effects such as papilledema and sixth nerve palsies, which can affect vision 2.
Treatment and Management
- The combination of ATRA and ATO has been shown to be effective in treating APL, with a high complete remission rate and low relapse rate 3, 4.
- The addition of gemtuzumab ozogamicin (GO) to ATRA and ATO may be beneficial in high-risk patients 4.
- Maintenance therapy with ATRA, with or without low-dose chemotherapy, is important for preventing relapse 5.
- Arsenic trioxide is considered the treatment of choice for relapsed patients 5.
Prognosis and Outcome
- The prognosis for APL patients has improved significantly with the use of ATRA and ATO, with a cure rate of over 80-90% 6.
- However, challenges remain, including early death and coagulopathy, particularly in high-risk patients 6.
- Ongoing efforts are focused on improving outcomes for high-risk and relapsed patients, and reducing the early death rate 6.