Roth spots show as initial presentation in multiple myeloma

October 20, 2016

Roth spots are hemorrhages in the retina with white centers. They occur in a wide variety of systemic conditions that are associated with retinal hemorrhages.

Roth spots are hemorrhages in the retina with white centers. They occur in a wide variety of systemic conditions that are associated with retinal hemorrhages.1 (See Table 1.) They can present a diagnostic challenge for the optometric physician because there are many considerations in the differential diagnosis.

The following case report demonstrates one such presentation.

Roth spots case report

A 57-year-old male presented as a new patient to the Mountain Home Veterans Affairs (VA) Medical Center optometry clinic needing an updated contact lens prescription. He successfully wore contact lenses for distance only. He used over-the-counter (OTC) reading glasses for near work and reported that he was happy with this mode of operation.

He stated that he had been to another office in the area for the last 15 years to get his contact lenses, and he complained that the other doctor hadn’t examined his eyes in a few years. He said the office checked that his vision was good and re-ordered the same contact lenses for him. He was concerned about the health of his eyes.

His review of systems was significant for hyperlipidemia, history of bilateral inguinal hernia, arthralgia of the knees, and a recent episode of pleurisy. He denied a history of diabetes.

His active medications were hydrocodone/acetaminophen as needed, sildenafil (Viagra, Pfizer) as desired, one baby aspirin daily, and fish oil daily (he didn’t know the amount).

He drove a truck for a living, used a computer regularly, and was a former cigarette smoker.

Other than his contact lens history his ocular history was unremarkable. He wore his contact lenses on a daily wear basis and used ReNu (Bausch + Lomb) solution to clean and disinfect his lenses daily.

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He brought his contact lens prescription with him; he was wearing:

OD: Acuvue 2 (etafilcon A, Johnson & Johnson Vision Care), 8.7 mm base curve, -3.00 DS

OS: SofLens Toric (alphafilcon A, Bausch + Lomb), 8.5 mm base curve, -2.00-1.25x090

He claimed to replace them once per month. His last eye exam was one year ago. He used +1.00 D OTC readers over his contact lenses for near.

His entering visual acuities with his contact lenses were OD 20/30 and OS 20/20-2. His best visual acuity with a spectacle refraction was OD 20/25 and OS 20/20.

His pupils were equal, round, and reactive to light without an afferent pupillary defect. Extraocular motilities were full and smooth without restrictions in both eyes. His confrontation visual fields were full to finger counting in each eye. Applanation tonometry was OD 11 mm Hg and OS 11 mm Hg at 8:29 a.m.

Slit-lamp biomicroscopy revealed mild meibomian stasis of the lids, mild and patchy punctate epithelial erosions on the corneas, and immature nuclear and cortical cataracts. Otherwise the anterior segment was unremarkable.

A dilated fundus examination revealed 0.6/0.6 cup in the right optic nerve and 0.7/0.7 cup on the left optic nerve. Neuroretinal rim tissue appeared to be pink and intact. I noted a couple choroidal nevi temporal to the macula in the right eye. However, the most remarkable finding was the numerous Roth spots in the posterior poles of each eye (Figures 1 and 2).

Spectral domain OCT of the nerve fiber layers of each eye indicated that the patient had nerve fiber layer thinning superiorly greater than inferiorly in the right eye and inferiorly greater than superiorly in the left eye.

After observing the numerous Roth spots in his posterior pole, we asked him additional history questions. When asked if he was fatigued, he stated that he had been a little tired lately, but that he attributed it to his seasonal allergies. At the time it was December in the mountains of east Tennessee, so seasonal allergies are usually at a relatively low point at that time of year. Because he was a truck driver, we was asked if he had been sitting or sleeping in his truck while idling. He hadn’t driven it in four days. He denied feeling ill, dizzy, or nauseated and any chills, shortness of breath, or chest pain.

Related: Contact lenses and dry eye: Cause or remedy?

 

Additional testing revealed cool and clammy skin. He was obviously afebrile but seemed a little cooler to the touch than expected. His blood pressure was 130/75 mm Hg.

Palpation of the preauricular and submandibular nodes was negative. Palpebral conjunctivae, lips, and fingernails all appeared to be of normal color. His mental status was alert and oriented to person, place, and time. He did not appear to be confused, and his mood was pleasant.

His fundus photos were explained to the patient and his wife, and they were told what Roth spots were. It was explained that there could be many different reasons for them, and it was necessary to perform a blood test to see if there was something going on that needed to be treated.

A stat complete blood cell count was arranged. Within 20 minutes, a lab technician called to report that the patient’s hemoglobin was critically low at 6.2 (the reference range for our hospital at that time was 13.6-17.3). We called the patient’s primary-care provider, and the patient was admitted to the hospital for severe anemia.

He was eventually diagnosed with multiple myeloma and started on chemotherapy to suppress his bone marrow for a bone marrow transplant. During the work-up for his anemia, kidney failure as a complication of the multiple myeloma was uncovered.

Sadly, the patient died of kidney failure merely six months after the Roth Spots were discovered.

Discussion

Roth spots were first noted in 1872 by Moritz Roth, a pathologist at the University of Basel.1,2 He reported a condition he called retinitis septica in patients who had been afflicted with bacteraemia. Roth assumed that the white centers in these hemorrhages represented bacterial abscesses.3

Litten reported in 1878 that these hemorrhages with white centers occurred in 80 percent of cases of subacute bacterial endocarditis.4 He was the first to give them the name “Roth spot,” and he noted that they appear and disappear quite rapidly.

About 90 years later, investigators noticed a remarkable absence of bacteria and leukocytes and instead found fibrin in the center of these hemorrhages in patients with aplastic anemia.5 Ling and James postulated that the white center may result from the platelet-fibrin thrombus that forms following a breach, or rupture, in the capillaries.1 They argue that this more commonly occurs in acute systemic changes than in diseases that cause more gradual changes such as diabetes and hypertension.

Related: The case of the disappearing drusen

 

We now know that there are many different etiologies for Roth spots. The differential diagnosis should be refined and developed after a detailed history and review of systems. Table 2 associates the various symptoms with many of the diagnoses from Table 1.

Based upon a thorough review of these symptoms, the optometric physician should narrow down the hypothesis, or differential diagnoses. Clinical correlation is key. Therefore, the differential diagnoses should be further refined by additional examination techniques. And many of the systemic etiologies require testing to be performed outside the optometrist’s office (Table 3).

Conclusion

The diagnostic strategy for Roth spots is very similar to working up a patient with recurrent or bilateral non-traumatic uveitis. A complete review of systems is very important, followed by additional testing to rule out the various associated systemic conditions. Due diligence on the part of the clinician pays off when the etiology is discovered. It is also extremely important to communicate findings with the patients’ primary-care physician to coordinate care.

References

1. Ling R, James B. White-centred reinal haemorrhages (Roth Spots). Postgrad Med J. 1998 Oct;74(876):581-2.

2. Roth M. Uber netshautuffecstionen bei wondfiebrin. [Retinal manifestations of wound fever] Deutsch A Chir. 1872;1:471-84.

3. Roth M. Beitrage zur kenntnis der varicosen hypertrophie der nervenfasern. [Contributions to the knowledge of varicose hypertrophy of nerve fibers.] Virshow’s Arch Path Anat. 1872;55:197-217.

4..Litten M. Ueber akute maligne endocarditis und die dabei vorkommenden retinal veranderungen. Charite-Ann. 1878;3:135.

5. Wong VG, Bodey GP. Haemorrhagic retinoschisis due to aplastic anaemia. Arch Ophthalmol. 1968 Oct;80(4):433-5.

6. Blumenthal EZ, Zamir E. Images in cardiovascular medicine. Roth's spots. Circulation. 1999 Mar 9;99(9):1271.

7. Medical Zone. Differential Diagnosis of Roth’s Spot. Available at: http://www.medicalzone.net/differential-diagnosis-of-roths-spot.html. Accessed 11/18/15. 

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