Hematological Disorders:
Ocular changes may indicate
underlying systemic condition
Sherrol A. Reynolds OD, FAAO
I.
Anemia
a. Pathophysiology- reduction of hemoglobin concentration or RBC’S
b. Types:
i. Iron Deficiency Anemia
 Most common Anemia
a. Iron is essential for Oxygen transport, DNA synthesis,
electron transport
 Causes: Malabsorption or blood loss
 Lab testing:
a. Serum iron (Fe), Transferrin (total iron-binding
capacity (TIBC), Serum Ferritin
ii. Vitamin B12 Deficiency (Pernicious Anemia)/ Folate deficiency
 lack intrinsic factor-needed to absorb vitamin B12 from GI
iii. Apastic Anemia
 Bone Marrow Failure
iv. Hemolytic Anemia
v. Anemia of Chronic disease
c. Clinical Presentation
i. signs and symptoms
d. Case presentation
i. Anemic Retinopathy
 hemorrhages, CWS,
 dilated & tortuous vessels, exudates,
 Roth spots
e. Management
II.
Sickle cell disease
a. Pathophysiology- an autosomal recessive genetic disorder
i. Sickle shape of RBC’s in response to decrease O2 tension
 Hypoxia, acidosis, and ischemia
b. Prevalence
c. Types:
i. Sickle cell anemia (SS)
ii. Sickle trait (SA)
iii. Sickle Thalassemia (S-thal)
iv. SC disease
d. Laboratory
i. Sickledex: in-office screening
e. Electrophoresis- confirm the diagnosis and type of disease is present
f. Clinical manifestation:
i. Anterior segment
ii. Non-proliferative retinopathy
 Dark Without Pressure
 Venous tortuosity of the peripheral vessels
 Salmon patch hemorrhage: intraretinal hemorrhage
 Black sunbursts
 Refractile deposits
iii. Proliferative neovascularization
 5 stages of proliferative sickle retinopathy:
I.
Arteriolar occlusion-Between ora and equator
II.
A-V anastomosis to bypass occlusion
III.
Surface neo and sea fans
IV.
Vitreous hemorrhage
V.
Tractional retinal detachment
g. Case Presentation
h. Treatment
i. Non-proliferative Retinopathy
 Observation for non-proliferative
ii. Proliferative Retinopathy
 Scatter photocoagulation
 Anti-VEGF
iii. Avoid CAI's (Acetazolamide) in glaucoma patients
iv. Landmark Bone-marrow treatment
III.
Lymphomas
a. Pathophysiology
i. Abnormal proliferation of T or B lymphocytes
b. Classification
i. Hodgkin or Non-Hodgkin
ii. Multinucleated, giant cells called Reed-Sternberg cells.
c. Clinical features
i. Lymphadenopathy
ii. Enlarge spleen
iii. Bone marrow filtration
d. Case presentation
e. Management
IV.
Leukemia
a. Pathophysiology
i. Disorder of blood-forming tissue characterized by:
 an abnormal proliferation of one of the WBC types
a. cells remain immature and never stop dividing
b. Etiology-A high incidence in persons exposed to:
i. high levels of radiation, certain toxins, retrovirus (human T-cell
leukemia), and chromosomal abnormalities
 Most cases have no clear cause
c. Types:
i. Acute: ~90% achieve remission with treatment (Cure up to 60%)
 Acute myelogenous leukemia-AML
 Acute lymphoblastic leukemia-ALL
ii. Chronic:
 Chronic myelogenous leukemia-CML
 Chronic lymphocytic leukemia-CLL
a. marked elevated WBC with mature forms dominating,
(+) Philadelphia chromo
d. Case presentation
i. Leukemic retinopathy
 preretinal and intraretinal hemorrhages
 Roth’s spots (leukemic infiltrate), cotton wool spots,
 retinal venous tortuosity and neovascularization
e. Management
i. chemotherapy
ii. Radiation
iii. Allogeneic bone marrow transplantation