Acute Myelogenous Lymphoma
Masquerading as VKH Disease
Nikolas London, MD
Retina Consultants San Diego
Sunir Garg, MD, FACS
MidAtlantic Retina
Ocular History
 21 year-old athletic white man with 1 week of
blurred vision in both eyes
 Past Ocular History: none
 Past Medical History: recent tonsillitis
 Social History: College student, active weightlifter
First Presentation – outside
ophthalmologist
 VA: OD 20/40, OS 20/40
 No anterior or vitreous cell
 Fundus:
 Bilateral, multifocal serous retinal detachments
Foveal OCT
OD
OS
Macular SDOCT revealing serous subretinal fluid in both eyes.
Note that EDI was not obtained at presentation.
First Presentation
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Diagnosed with VKH Disease
Started on prednisolone 60 mg daily
No improvement
Developed intermittent face and jaw paresthesias,
mild fatigue, mild bruising of his arms, and several
mouth ulcers, all attributed to the prednisone
First Presentation
FLA and ICG
 Multifocal pinpoint areas of
hyperfluorescence within the areas of
serous retinal detachment with no
evidence of choroidal perfusion
abnormalities
Presentation to us 1 month after onset
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Alert and oriented
BP 163/77
Unchanged exam
WBC of 327,000 cells/uL
Sent immediately to ER
Hospital Course
 Evidence of DIC
 Fibrinogen 61 mg/dL (140-476)
 D-dimer 12.3 ug/mL(< 0.5)
 Multifactorial respiratory failure
 Acute renal failure
 Bacteremia
 Leukemia cutis
 Subconjunctival hemorrhage
 Small intracranial hemorrhage
 Pleural effusions and leukemic infiltrates
Bullous subconjunctival hemorrhages
shortly after presentation
Leukemia cutis
Leukemia cutis
Sagittal Head CT
 small area of subacute
hemorrhage in the right
posterior fronatl lobe
with minimal
surrounding edema
Chest CT
Pulmonary leukemic
infiltrates,
small bibasilar
effusions,
 multiple lymph nodes
Bone Marrow Biopsy
 markedly hypercellular, demonstrating blasts that
displayed irregular nuclei, immature chromatin, and
scant-to-moderate cytoplasm
Peripheral Blood Smear
 markedly increased number of abnormal and immature
monocytoid cells consistent with AML
Treatment
 Started on systemic chemotherapy,
including idarubicin and cytarabine 7 + 2
 Bone marrow transplantation.
Follow-up – 1 week after admission
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VA improved to 20/30 OD and 20/40 OS
The subconjunctival hemorrhages were resolving
The serous retinal detachments had resolved
Fundus: few retinal hemorrhages in both eyes and a
single cotton-wool spot in the right eye
 Enhanced depth imaging OCT revealed a prominently
thickened choroid OU
Follow up – 1 week after administration
Few retinal hemorrhages, OD cotton-wool spot
Serous retinal detachment has resolved
Enhanced Depth Imaging OCT
 Prominently thickened choroid
 May result from leukemic
infiltration or/and vascular
congestion
OD
OS
Final Diagnosis
 Leukemic choroidopathy
 Resolution with treatment of underlying
disease
Conclusion
 Serous retinal detachment is a rare manifestation
of acute myelogenous leukemia that can mimic
VKH or multifocal CSCR
 Accurate diagnosis and management can be lifesaving
 Clinicians should maintain a high index of
suspicion of a hematologic malignancy in cases
of “atypical” VKH or CSR and consider a CBC