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Hematological disorders can manifest in the anterior, posterior segment
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Anemias, sickle cell disease, hematological malignancies and platelet disorders can all cause ocular complications, and optometrists should be cognizant of these specific symptoms.
“The biggest thing is for optometrists to make sure the patient gets the proper medical evaluation and workup, so we can get the proper diagnosis and prescribe the proper treatment,” Sherrol A. Reynolds, OD, FAAO, said in an interview with Primary Care Optometry News.
A thorough evaluation, Reynolds added, is also important because, as with anemic retinopathy, the ocular complication may be reversed once the underlying cause is treated.
Ocular manifestations
“These hematological disorders can present from the front to the back of the eye, as well as cause visual disturbance, such as in visual acuity, visual aberration and vision loss,” Reynolds said.
Anterior segment signs that could indicate a hematological disorder include changes in conjunctival vasculature, a change in the color of the conjunctiva and changes in the iris, such as iris atrophy and iris neovascularization, Reynolds said.
Posterior segment signs can include retinopathy, maculopathy, intraretinal hemorrhages, exudates, ischemia, retinal detachment, retinal exudative changes and optic neuropathy, she said.
“Some cases of hematological malignancy might present with a swollen optic nerve head,” Reynolds said. “You might have a conjunctival lesion if the patient has lymphoma. You can have leukemic plaque on the front of the eyes, proptosis of the eyes, palsies due to anemia.”
Sherrol A.
Reynolds
A careful history and CBC with differential is crucial, she said.
Anemia
There are many types of anemias, ranging from vitamin-deficient to hemolytic, the causes of which may include malabsorption, blood loss, genetics or chronic diseases. All will have ocular manifestations that may include conjunctival jaundice, changes in pallor of the optic nerve, disc swelling, retinal hemorrhages, cotton-wool spots, dilated and tortuous blood vessels, exudates and Roth’s spots. Reynolds explained in a lecture at the American Academy of Optometry annual meeting that these ocular conditions are caused by hypoxia or ischemia.
Nonocular signs and symptoms can include fatigue or weakness, dizziness, headaches, shortness of breath, numbness or coldness in fingers and toes, pallor of the skin and nail beds, brittle nails, and a fast or irregular heartbeat, she told attendees.
If a suspicion of anemia exists, the clinician should order a CBC with differential to rule out underlying systemic diseases. Once the anemia is determined, the clinician should treat the specific type of anemia and follow a conservative mode of management with 3-month follow-up.
“Anemic retinopathy is always reversible with correction of the anemia,” Reynolds said.
Sickle cell disease
Sickle cell disease is an autosomal recessive genetic disorder that results in normal hemoglobin becoming rigid and crescent-like, or sickle, in shape in response to low oxygen tension. The blood can sickle in response to deoxygenation, dehydration and acidosis and becomes more viscous and less able to carry oxygen, which can result in hypoxia, ischemia, hemolysis and vaso-occlusive events that can cause tissue and organ damage, Reynolds said.
Manifestations of sickle cell disease include comma- or S-shaped capillary segments in the bulbar conjunctiva, focal iris atrophy, iris neovascularization, subconjunctival hemorrhages, spontaneous hyphemas, rubeosis iridis, secondary neovascular glaucoma, sea fan neovascularization and sickling maculopathy, she said.
If sickle cell disease is suspected, the clinician should order Sickledex (Streck), a solubility test that detects the presence of hemoglobin S or, more useful, a hemoglobin electrophoresis. A DNA analysis can also be ordered, Reynolds said.
Sickle cell retinopathy is classified as nonproliferative or proliferative. Clinicians can manage patients with nonproliferative retinopathy without referring and should follow up with them every 3 to 6 months, she said.
Proliferative retinopathy is more complex. In the early stages clinicians can follow the same action as they would for nonproliferative retinopathy. In the later stages, patients may require laser photocoagulation or retinal surgery, and so may need to be referred to a retinal specialist, she said.
Sickle cell trait
Although sickle cell disease is one of the most common hematological disorders clinicians see, sickle cell trait may be the more dangerous of the two, due to its facility for flying under the radar, according to Reynolds.
“Usually when we hear the term sickle cell trait, we generally think of that as a benign condition,” she said in the interview with PCON. “These patients aren’t sick — they won’t have retinopathy and they won’t have the systemic complications associated with sickle cell disease. However, patients that have comorbidity, such as unusual high blood pressure, inflammatory disease or lupus, are more at risk for having episodes where their trait converts to sickle cell anemia.
“We hear quite a bit about renowned athletes passing away suddenly,” Reynolds continued. “They were practicing and all of a sudden they fell ill and died. A lot of these patients, particularly those of African-American descent, tend to have undiagnosed sickle cell trait. While exercising and working out, they can have an episode where, because of the hypoxia and the stress on the system, their sickle cell trait converted to sickle cell and caused other problems. That’s why we’ve called it the silent killer.”
Peripheral retinal changes such as these may be associated with sickle cell retinopathy.
Image: Reynolds SA
Some risk factors for the sickling that occurs in patients with sickle cell trait include a change in altitude, heat stress, rapid conditioning and sustained maximal exertion, according to Reynolds’s academy lecture.
Leukemia
Ocular complications associated with leukemia include lacrimal gland infiltration, extraocular muscle infiltration, eyelid swelling, leukemic plaque, subconjunctival hemorrhages, petechial hemorrhages, chemosis and pain, exudates, cotton-wool spots, retinal vein tortuosity and dilation, peripheral retinal microaneurysms, retinal neovascularization, direct infiltration of the optic nerve head or the papilledema, Reynolds told academy attendees.
Management options for these patients can consist of systemic chemotherapy or direct radiation, injection of chemotherapeutic agents into the subconjunctival area or a bone marrow transplant, she said.
“If the patient has acute leukemia and isn’t treated rapidly, they generally don’t live very long. In those with chronic diseases, of course they need to be treated, but these patients generally do a little bit better,” she said. – by Daniel R. Morgan
References:
Reynolds SA. Hematological disorders and the eye: ocular changes may indicate underlying systemic conditions. Presented at: Academy 2012; October 2012; Phoenix, AZ.
For more information:
Sherrol A. Reynolds, OD, FAAO, is an associate professor at Nova Southeastern College of Optometry, Fort Lauderdale, Fla. She can be reached at (954) 262-1442; sreynold@nova.edu.
Disclosure: Reynolds has no relevant financial disclosures.