CASE REPORT

Rapidly progressing bilateral cataracts in a patient

with beta thalassemia and pellagra
Ioannis Athanasiadis, MD, MRCSEd, Aristeidis Konstantinidis, MD, MRCOphth,
Ioannis Kyprianou, MRCOphth, Rosemary Robinson, FRCSI, FRCOphth,
Vaia Moschou, MD, PhD, Kokkona Kouzi-Koliakos, MD, PhD

Soon after the diagnosis of pellagra in a 20-year-old patient with beta thalassemia, bilateral intu-
mescent cataracts rapidly developed. We believe the patient’s crystalline lenses were at an in-
creased oxidative state due to iron overload from the thalassemia. Depletion of the lens
epithelial cells of an important antioxidative agent (glutathione) as a result of niacin (vitamin
B3) deficiency due to pellagra reduced the antioxidative capacity of the lenses. The oxidative dam-
age led to rapid development of cataracts.
J Cataract Refract Surg 2007; 33:1659–1661 Q 2007 ASCRS and ESCRS

Pellagra is a systemic disturbance caused by a cellular
deficiency of niacin, resulting from inadequate dietary
nicotinic acid and/or its precursors. Patients with beta
thalassemia often have mild lenticular opacities.1
Previous authors suggest that chelation therapy (des-
ferroxamine) may play a protective role against these
changes.2 We present a case of rapidly progressive
bilateral intumescent cataract formation in a patient
with beta thalassemia and nutritional deficiency
and postulate the mechanisms implicated in the
pathogenesis.
CASE REPORT
A 20-year-old woman with beta thalassemia was referred to
our clinic by the hematologists due to bilateral reduced
visual acuity for 3 weeks. Pellagra had recently been clini-
cally diagnosed. However, no obvious cause was identified
as the medical history was otherwise unremarkable.
When the patient presented to the ophthalmology unit,
she was on systemic treatment that included niacin, vitamin
B complex, and a diet rich in calories, which had improved
the general symptoms, and also desferroxamine, which
kept the iron status stable. The uncorrected visual acuity
(UCVA) was 6/12 in both eyes and did not improve with
glasses or pinhole. Mild cortical opacities were evident in
both eyes.
No decision for cataract surgery was made on the first
visit. One week later, the patient returned because the symp-
toms had worsened. The UCVA was 6/36 in both eyes and
did not improve with glasses or pinhole. The lenses showed
further diffuse opacification and signs of intumescence. The
patient agreed to proceed with bilateral cataract extraction as
she was not coping well with daily activities. She had been
told that the lens changes were irreversible. A-scan biometry
was performed and showed average ocular dimensions in
both eyes (Figure 1, a).
One week later, the preoperative evaluation showed sig-
nificant intumescence and opacification of both lenses (Fig-
ure 2). The UCVA was 6/60 in both eyes, not improving

Accepted for publication May 4, 2007.

From the Ophthalmology Department (Athanasiadis, Konstantini-
dis, Kyprianou, Robinson), Walsgrave Hospital, Coventry and War-
wickshire University Hospitals NHS Trust, Coventry, United
Kingdom; and the AHEPA University Hospital (Moschou, Kouzi-
Koliakos), Aristotle University of Thessaloniki, Greece.

No author has a financial or proprietary interest in any material or

method mentioned.
Corresponding author: Dr. I. Athanasiadis, Walsgrave Hospital,
Ophthalmology Department, Coventry and Warwickshire University
Hospitals NHS Trust, Clifford Bridge Road, Coventry, CV2 2DX
United Kingdom. E-mail: athana1972@yahoo.com.
Figure 1. a: Initial A-scan of the right eye. b: A-scan of the same eye 1
week later. Note the signs of intumescence with concurrent shallow-
ing of the anterior chamber and the vitreous cavity. Crystalline lens
thickness increased by 2.46 mm, while the anterior chamber depth
decreased by 0.35 mm.

Q 2007 ASCRS and ESCRS 0886-3350/07/$dsee front matter 1659

Published by Elsevier Inc. doi:10.1016/j.jcrs.2007.05.011
1660 CASE REPORT: BILATERAL CATARACTS WITH BETA THALASSEMIA AND PELLAGRA

Figure 2. Photograph of the left eye showing signs of crystalline lens Figure 3. Disruption in the cytoplasm of a lens fiber (black arrow).
intumescence and shallowing of the anterior chamber.

with glasses or pinhole. A new A-scan ultrasonography

showed a remarkable increase in lens thickness with a reduc-
tion in the anterior chamber depth and vitreous cavity in
both eyes (Figure 1, b). This prompted a decision for early
surgery.
Following uneventful phacoemulsification, the patient’s
UCVA improved to 6/6 bilaterally. The intraocular pressure
was within normal limits in both eyes throughout the clinical
course, ranging between 10 mm Hg and 13 mm Hg. Lens
fragments were sent for histological examination. Electron
microscopy revealed structural damage of the lens fibers sec-
ondary to oxidative stress (Figures 3 and 4).

DISCUSSION
Pellagra is a metabolic condition that results from nia-
cin (vitamin B3) deficiency. In this case, the diagnosis
of pellagra was based on the patient’s history and
the presence of the 3 D syndrome: dermatitis, diarrhea,
and dementia.
Vitamin B3 plays a role in glutathione synthesis, the
most important antioxidant of the crystalline lens.3,4
Our patient also suffered from beta thalassemia. It is
known that patients with this condition undergo high-
er oxidative stress due to the excess iron load.5,6 We
suggest that the crystalline lenses in our patient were
at an increased oxidative state. Pellagra-induced deple-
tion of the glutathione levels renders the crystalline
lenses susceptible to oxidative damage. The cataracto-
genesis seemed to be due to free radical damage as a re-
sult of the iron excess. This is supported by the results
of electron microscopy of the cataractous lenses. Simi-
lar findings have been reported in studies in rats.7
In the case of our patient, the cataracts developed
soon after the clinical manifestation of pellagra. The in-
terval between the clinical diagnosis of pellagra and
the cataract formation as well as the bilaterality of
the condition suggest a causative relationship. To
our knowledge, there is no similar report in the
Figure 4. Lens fibers are elongated, and the cell membrane has irreg-
ular protrusions that interdigitate with those of the adjacent cells
(small arrows). The cytoplasm was occupied by fine granular mate-
rial, and no organelles were observed (large arrows). Some fibers ap-
peared disrupted in the cytoplasm, and high stained membranes
were present in the area (arrowheads).
literature. However, there are clinical studies confirm-
ing the protective role of vitamin B complex and other
agents against the development of cataract.8–10
This case highlights the importance of antioxidants
and a balanced diet in the metabolic stability of the
lens. We believe that rapidly progressing lenticular
opacities in subjects with beta thalassemia may be
related to nutritional deficiencies.
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