Commented [

SYSTEMIC DISEASES

Presenters
MAGABIRO DAVID
NELSON PONERA

INTRODUCTION
• Leukemia

• Lymphoma

• Anemia

• Sickle cell disease

HEMATOPOIESIS
 LEUKEMIA
• Cancer of white blood cells

• Unlike others

• What really happens?

• Depending on lineage(myeloid or lymphoid)

CLASSIFICATION OF LEUKEMIA
SYSTEMIC PRESENTATION
 OCULAR MANIFESTATIONS
• Proptosis ;leukemic deposits in the orbital tissue

• Leukemic retinopathy common to both

Pale and orange fundus background
Dilated and tortuous veins
>>greyish white lines

Arterioles pale and narrow

Roth’s spots
Large pre-retinal(sub-hyaloid) hemorrhages
 OTHER OCULAR MANIFESTATIONS
• Rare involvement of cornea, sclera and conjunctiva

• Usually secondary to anemia, thrombocytopenia and opportunistic infections

• Orbital infiltration into lacrimal gland, drainage system, rectus muscle and dermis

• Resembles orbital cellulitis in children

TREATMENT
• Depending on type of leukemia
• Systemic chemotherapy

• radiation

LYMPHOMA
• Another blood cell tumor

• Specifically

• Either Hodgkin or non-Hodgkin’s

• Either B or T cells
 RISK FACTORS
• Age

• Viral infections

• Immunosuppressions

• Sex

• Genetics and autoimmune diseases

SYSTEMIC PRESENTATION
• Lymphadenopathy
 • B symptoms

• Accompanied with
• Loss of appetite
• Fatigue
• Chest pain, cough, respiratory distress
• Back pain
• pruritus

PCNSLO
• Primary CNS lymphoma with ocular involvement

• Arises outside the CNS and metastases into the eye

• More common in Non-Hodgkin’s lymphoma

• PCNSLO more common than systemic metastasis.

OCULAR MANIFESTATIONS
• Lid and orbital deposits
90% bilateral
Painless decrease or LOV
Red eye
Photophobia
Diplopia

• Intra-orbital
Primary iridocyclitis and psudohypopyon
 OCULAR MANIFESTATIONS
• On fundoscopy
Low lying yellow to white mass deep to the sensory retina
May be infiltrative of all layers
Rarely hemorrhage

• Deep infiltrates leads to retinal detachment

• With healing scarring is the only finding

• Optic neuropathy may be present

 OCULAR MANIFESTATION
Salmon pink appearance

Mass usually rubbery, firm without bony

Destructions

Lacrimal glands and EOM may be involved

MANAGEMENT
• Radiotherapy

• Intrathecal chemotherapy
• Combined chemoradiation

ANEMIA
• Decrease in amount of blood cells (RBCs) or hemoglobin
• Depending on age and sex
SYSTEMIC PRESENTATION
OCULAR EXAMINATION
 ANEMIC RETINOPATHY
• Disorder of retinal vasculature

• With Hb fall <50%, consistent with < 35%

• Usually asymptomatic

• But may present with visual loss

OPHTHALMOSCOPY FINDINGS
• Generalized pallor

• Retinal veins dilation

• Roth’s spots

• Soft retinal exudates

• Cotton wool spots

TREATMENT
Reversed with correction of Hb

• Treat the cause

• Medication

• Diet
 SICKLE CELL ANEMIA
• Hereditary form of anemia

• Mutated form of HbS distorts RBCs into crescent shape

• Sickled cells don’t pass easily through the small vessels

• Tendency of thrombosis and hemorrhage

OCULAR MANIFESTATION
ANTERIOR SEGMENT
• Comma shaped(cork screw) conjunctival vessels
• Cataracts

• Phthisis bulbi

• hyphema
OCULAR MANIFESTATION
 OCULAR MANIFESTATION
POSTERIOR SEGMENT
Can be grouped into 6:
i. Optic disc changes
ii. Posterior retinal and macular vascular occlusion iii. Chronic macular
changes (sickling maculopathy) iv. Choroidal vascular occlusions
v. Non proliferative retinal changes
vi. Proliferative retinal changes

OCULAR MANIFESTATION
 Posterior retinal & macular vascular occlusions; BRAO or CRAO.

Chronic macular changes

Micro aneurysms resembling dots
Hair pin-shaped vascular loops
Abnormal foveal avascular zone (FAZ)

OCULAR MANIFESTATION
• Choroidal vascular occlusions; Extremely rare.

• Non proliferative retinal changes

- Venous tortuosity
- Salmon-patch hemorrhage
 - Black sunburst
• Proliferative sickle retinopathy (PSR)

OCULAR MANIFESTATION
• Venous tortuosity due to arteriovenous shunting from the retinal periphery

• Salmon patch hemorrhage –superficial intraretinal hemorrhage(red to salmon color due to

hemolysis)

• Schisis cavity –space caused by disappearance of intraretinal hemorrhage

• Black sunburst consist of round chorioretinal scars at the equatorial fundus resulting from
 OCULAR MANIFESTATION

Salmon patch appearance

 OCULAR MANIFESTATION

Black sunburst
 OCULAR MANIFESTATION
• PSR PROLIFERATIVE SICKLE CELL RETINOPATH

• Chronic changes of local hypoxia and ischemia  leads to upregulation of VEGF(vascular

endothelial growth factor)  Neovascularization  pre retinal or vitreous hemorrhage
and tractional retinal detachment

OCULAR MANIFESTATION
• STAGES OF PSR
Goldberg classified PSR into five stages
3. Stage of neovascularization.
4. Stage of vitreous hemorrhage.
5. Stage of Retinal detachment

STAGES OF PSR
• In stage I, the peripheral arteriolar vessels occlude, early in the process the occluded arterioles are
dark red lines but eventually they turn into silver-wire–appearing vessels.

• In stage II, peripheral arteriolar-venular anastomosis occurs as the eye adjusts to peripheral
arteriolar occlusion and blood is diverted from the occluded arterioles into the adjacent venules
 STAGES OF PSR
• In stage III, new vessel formation occurs at the junction of the vascular and avascular retina. These
neovascular tufts resemble sea fans which initially are fed by a single arteriole and draining vessel

Later, as the sea fan grows in size, The sea fans may acquire a glial and fibrotic tissue envelope.
This envelope may pull on the vitreous. A full-thickness retinal break, which may lead to total
rhegmatogenous retinal detachment, may occur.

STAGES OF PSR
• Stage IV
Vitreous hemorrhage, Vitreous hemorrhage can result from PSR or trauma. It may be spontaneous
secondary to vitreous collapse and traction of the adherent neovascular tissue.
 Retinal detachment, Retinal detachments may be rhegmatogenous and/or tractional. Traction
from bands and membranes on the neovascular tufts can lead to sufficient retinal traction with
or without retinal tears
 STAGES OF PSR

Fluorescein angiogram showing sea fans

 STAGES OF PSR

Retinal detachment
 MANAGEMENT
EXAMINATION & INVESTIGATION

• VA
• IOP
• Fundoscopy
• Fluorescein angiography
• Optical coherence tomography
• Hemoglobin electrophoresis is of importance to confirm diagnosis
 MANAGEMENT
• Screening, prevention, early treatment to avoid complications

• The goal of treatment is to eliminate existing neovascularization and, thus, to eliminate the
sequelae of proliferative sickle retinopathy (PSR).

• Modalities to treat proliferative sickle retinopathy include diathermy, cryotherapy, pan

retinal photocoagulation(to regress neovascularization), and argon laser photocoagulation

MANAGEMENT
• Elevated intraocular pressure
 Blood in the anterior chamber in patients with sickle cell disease is a medical emergency. The
environment of the anterior chamber promotes sickle hemoglobin polymerization, which can
result in elevated intraocular pressure due to blockage of the trabecular meshwork.

• Pars plana vitrectomy is required for non clearing vitreous hemorrhage. It shouldbe
followed by repair of the retinal detachment, when present

REFERENCES
•, 4th edition, 2007
•http://retinatoday.com/2016/06/anemic-retinopathy-case-reports-and-diseasefeatures/
•UPtoDate
•Medscape
• Youtube khan academy videos