Group Title: Cases Journal 2008, 1:322
Title: Aplastic anemia induced disc edema and visual loss in pregnancy: a case report
Full Citation
Permanent Link:
 Material Information
Title: Aplastic anemia induced disc edema and visual loss in pregnancy: a case report
Series Title: Cases Journal 2008, 1:322
Physical Description: Archival
Creator: Gupta SK
Brar VS
Keshavamurthy R
Chalam KV
Publication Date: 39770
 Record Information
Bibliographic ID: UF00100189
Volume ID: VID00001
Source Institution: University of Florida
Holding Location: University of Florida
Rights Management: Open Access:


This item has the following downloads:

aplastic ( PDF )

Full Text

Cases Journal

BioMed Central

Case Report

Aplastic anemia induced disc edema and visual loss in pregnancy: a
case report
Shailesh K Gupta, Vikram S Brar, Ravi Keshavamurthy and
Kakarla V Chalam*

Address: University of Florida-College of Medicine, Department of Ophthalmology, Jacksonville, Florida, USA
Email: Shailesh K Gupta; Vikram S Brar; Ravi Keshavamurthy;
Kakarla V Chalam*
* Corresponding author

Published: 18 November 2008
Cases journal 2008, 1:322 doi: 10.1186/1757-1626-1-322

Received: 24 September 2008
Accepted: 18 November 2008

This article is available from:
2008 Gupta et al; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Introduction: A case of aplastic anemia diagnosed during pregnancy, which developed bilateral
disc edema and acute pre-retinal hemorrhage leading to vision loss.
Case Presentation: A 20 year old primagravid female developed acute vision loss in her right
eye, during hospitalization for treatment of aplastic anemia diagnosed during her pregnancy. Her
best-corrected visual acuity (BCVA) was hand motions and funds evaluation revealed a large pre-
macular hemorrhage in the right eye (OD) and bilateral disc edema. Neuro-imaging studies did not
reveal any signs of intracranial mass lesion or edema.
Conclusion: There was resolution of the disc edema with improvement in the pre-macular
hemorrhage resulting in 20/50 vision in the right eye, following supportive transfusions. Ophthalmic
manifestations developing in a pregnant patient with aplastic anemia can be successfully managed
with supportive care including red blood cell and platelet transfusions.

Aplastic anemia, a serious hematological disorder charac-
terized by pancytopenia and hypoplastic bone marrow is
often exacerbated during pregnancy [1-3]. Hormonal
imbalance between hematopoietic placental lactogen and
erythropoietin and marrow suppressive estrogen result in
this association [3]. We report an unusual case of bilateral
disc edema and visual loss due to pre-retinal hemorrhage
in a patient with aplastic anemia, diagnosed during preg-

Case presentation
A 20 year-old primagravid Caucasian female at 20 weeks
of gestation presented to the emergency department with

complaints of weakness, dizziness, headaches and palpi-
tations. Complete blood count (CBC) analysis revealed
white blood cell count (WBC) of 1.9 x 109 cells/litre,
hemoglobin of 4.9 gm/dl, and platelet count of 5 x 109
cells/litre. Further investigation showed normal liver func-
tion tests and prothrombin time and an INR of 13.3s and
0.9 respectively. Bone marrow biopsy confirmed the etiol-
ogy of the pancytopenia as aplastic anemia and subse-
quent red blood cell and platelet transfusions stabilized
the patient's hematologic status.

During her initial hospitalization, she reported decreased
vision in her right eye which she described as a red spot
when looking at the light. On ophthalmic consultation,

Page 1 of 4
(page number not for citation purposes)

her visual acuity was hand motions (HM) in the right eye
and 20/20 in the left eye, with normal intraocular (IOP)
in both eyes. Anterior segment examination was unre-
markable and pupillary reactions were normal. Dilated
fundoscopic examination done at that time had revealed
bilateral optic disc swelling and a layered pre-retinal hem-
orrhage involving the macula of her right eye (Figure 1).
Humphrey visual field examination (HVF) revealed
enlarged blind spots in both the eyes with a ceco-central
defect (mean deviation -4.02 db) in the right superotem-
poral quadrant, correlating with the retinal hemorrhage
present clinically (Figures 2A,C). Neuro-imaging, includ-
ing both CT and MRI, did not reveal a mass lesion and
lumbar puncture resulted in a dry tap in spite of repeated

The visual acuity in the right eye and the disc edema grad-
ually improved. During this course, the patient's hemato-
logic status and fetus were closely monitored, with
administration of supportive transfusions as needed. The
patient went on to deliver a 1664 gram infant at 32 weeks
gestation. On post-partum day 2, her visual acuity was 20/
50 and 20/20 in the right and left eyes, respectively.
Dilated fundoscopic examination revealed resolving pre-
retinal hemorrhage, clearing from the visual axis, with res-
olution of the disc edema bilaterally (Figure 3). Repeat
Humphrey visual field testing exhibited normalization of
the blind spot in both the eyes, with improvement in the
paracentral scotoma of the right eye (mean deviation -
2.63 db) correlating with the remaining pre-retinal hem-
orrhage (Figures 2B, D).

The first reported case of aplastic anemia was in a preg-
nant individual in 1888 [4]. Other conditions associated
with aplastic anemia include idiosyncratic drug reactions
(chloramphenicol), chemical exposure, eosinophilic fas-
ciitis, and seronegative hepatitis [5]. 78% of cases of aplas-
tic anemia exhibit ophthalmic manifestations. Typical
ophthalmic manifestations include eyelid hematoma,
subconjunctival hemorrhage, cotton wool spots, retinal
nerve fiber layer hemorrhage, Roth's spots, pre-retinal
hemorrhage, vitreous hemorrhage, and disc edema. Of
these, retinal hemorrhage and cotton wool spots are the
most common, 67% and 38% respectively [6].

Vision loss in our case is secondary to pre-retinal hemor-
rhage overlying the fovea in the right eye. Visual impair-
ment secondary to pre-retinal hemorrhages can be a
presenting symptom in previously undiagnosed cases of
aplastic anemia [6]. The presence of hemorrhages in the
setting of anemia and thrombocytopenia has been
described as part of the constellation of retinal findings in
patients with anemic-thrombocytopenic retinopathy.
Carraro et al reported an increased prevalence of retinop-
athy in patients with hemoglobin levels <8 mg/dL and
platelet counts <50 x 109/L, which is the case for many of
the patients affected by aplastic anemia. 2% of patients
with retinal hemorrhages exhibited pre-retinal lesions and
1 of 65 patients with ocular findings exhibited bilateral
disc edema [7].

Optic disc edema has been reported to occur in 6% of
cases of aplastic anemia, with the etiology most likely
being related to elevated intracranial pressure [6]. Two

Figure I
Color funds photograph depicting layered pre-retinal hemorrhage in the right eye and disc edema. Peri-papil-
lary nerve fiber layer hemorrhages are also present.

Page 2 of 4
(page number not for citation purposes)

Cases Joumnal 2008, 1:322

:::::::::::: I
i : ::: : : : : ? : i : :
: : : : :: '. `. i i: i i :
i:: :: : ::'.~.' .::: :


SU: *..



: : : : : : : :
: : : : : : :: : : : : i
: :: :: : : : ii : : : :
: ; i: i :: : i : :: i i i :
: ii : : : : :: : : ::: : : : :
: : i i : : : :t :: : : : :: : : : :
: : : : : i : :: : '
: :::::b:l::~ .

I 128i


Figure 2
(A) 24-2 Humphrey visual field (HVF) revealing enlarged blind spot with a superior paracentral scotoma in the
right eye (mean deviation -4.02 db). (B) Follow up 24-2 HVF demonstrating normal blind spot and improved superior
paracentral scotoma in the right eye (mean deviation -2.63 db). (C) 24-2 HVF revealing enlarged blind spot in the left eye
(mean deviation -2.79 db) (D) Follow up 24-2 HVF demonstrating normal blind spot (mean deviation (-0.74 db).

separate case reports describe the occurrence of idiopathic relationship by describing 7 cases of improvement of
intracranial hypertension in 3 adolescent patients with papilledema associated with anemia by correction of ane-
aplastic anemia, which responded to management with mia alone. In another case from the same series, the
acetazolamide and correction of the anemia [8,9]. A patient's papilledema only responded to the correction of
recent retrospective case series further highlighted this the anemia despite prior interventions to lower the intrac-

Page 3 of 4
(page number not for citation purposes)

A .::


| | I

AA.1 31A. I ),A_%

Cases Joumnal 2008, 1:322

. . . . . .:

. . .....::

: :::::~.
: :::::.


i: : : : : : : :: ::: i : i i : : ;
i : : : : i :: i i :: : : : : i i

Figure 3
Color funds photograph showing resolved disc edema in both eyes and central clearing of the pre-retinal
hemorrhage in the right eye.

ranial pressure [10]. In our patient, we could not establish
raised intracranial pressure as the cause for optic disc
edema as the lumbar puncture resulted in a dry tap. None
the less, our patient's disc edema and enlarged blind spot
on HVF testing gradually improved as the hemoglobin
levels stabilized.

In summary, this report illustrates a case of visual loss
associated with pregnancy related aplastic anemia which
was successfully managed with supportive care including
red blood cell and platelet transfusions.

CBC: Complete blood count; BCVA: Best-corrected visual
acuity; WBC: White blood cell count; IOP: Intraocular
pressure; CT: Computed Tomography; MRI: Magnetic Res-
onance Imaging.

Written informed consent was obtained from the patient
for publication of this case report and any accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.

Competing interests
The authors declare that they have no competing interests.

Authors' contributions
KVC and SG identified the case and directly participated
in management. They also revised the manuscript and ver-
ified its intellectual content. VSB and RK worked in collab-

oration to collect data, acquire clinical photographs, and
draft, revise, and reference the manuscript.

I. Cohen E, Ilan Y, Gillis S, Dann EJ, Rachmilewitz EA: Recurrent bone
marrow hypoplasia associated with pregnancy. Acta Haematol
1993, 89(I):32-4.
2. Snyder TE, Lee LP, Lynch S: Pregnancy-associated hypoplastic
anemia: A review. Obstet Gynecol Surv 1991, 46:264-269.
3. Choudhry VP, Gupta S, Gutpa M, Kashyap R, Saxena R: Pregnancy
associated aplastic anemia- A series of 10 cases with litera-
ture review. Hematology 2002, 7(4):233-238.
4. Ehrlich: "Uber einen fall von Anamie, mil Bemer Kungen uber
regenerative veranderungen des knochen marks". Charite Ann
1988, 13:300-309.
5. Young NS: Pathophysiologic mechanisms in acquired aplastic
anemia. Hematology Am Soc Hematol Educ Program 2006:72-7.
6. Mansour AM, Salti HI, Han DP, Khoury A, Friedman SM, Salem Z,
Ibrahim K, Bazerbachi A, Saqhir N: Ocular findings in aplastic
anemia. Ophthalmologica 2000, 214:399-402.
7. Carraro MC, Rossetti L, Gerli GC: Prevalence of retinopathy in
patients with anemia and thrombocytopenia. Eur] Haematol
2001, 67:238-244.
8. Jeng MR, Rieman M, Bhakta M, Helton K, Wang WC: Pseudotumor
cerebri in two adolescents with acquired aplastic anemia. J
Pediatr Hematol Oncol 2002, 24(9):765-8.
9. Nazir SA, Siatkowski RM: Pseudotumor cerebri in idiopathic
aplastic anemia. AAPOS 2003, 7(1):71-74.
10. Biousse V, Rucker JC, Vignal C, Crassard I, Katz BJ, Newman NJ:
Anemia and papilledema. Am) Ophthalmol 2003, 135:437-446.

Page 4 of 4
(page number not for citation purposes)

Cases Joumal 2008, 1:322

University of Florida Home Page
© 2004 - 2010 University of Florida George A. Smathers Libraries.
All rights reserved.

Acceptable Use, Copyright, and Disclaimer Statement
Last updated October 10, 2010 - - mvs