An Unexpected Surprise: Retinal Hemorrhage and Acute Leukemia
A 50 year old female presents to the ED for a spot in her vision. She woke up with an “ink splotch” in her right eye, located just off the center of her vision. She is sent from urgent care for ophthalmology evaluation. She denies pain, trauma, flashes, and floaters. She endorses intermittent headaches and nausea, and increased fatigue over past few weeks which she attributes to being peri-menopausal. Her PMH is consistent for hypothyroidism, for which she is taking Synthroid. She used to be a smoker but quit 20 years ago. Family history is insignificant.
Vitals: T 36.8, HR 67, BP 153/89, SpO2 98%, RR 16
Well appearing female
Non-focal neurological exam, no cerebellar findings, normal gait
No skin findings, no lymphadenopathy
Visual acuity OD 20/25 OS 20/70 (baseline)
IOP OD 16, OS 14 (normal)
Visual fields intact to confrontation
No conjunctival erythema/edema
Fundus exam – OD + retinal hemorrhage
WBC 28.3, Hgb 6.0, Hct 18, Platelet count 35
Manual differential showed 76% blasts
Na 140, K 3.5, Cl 103, Bicarb 27, Glucose 127, Cr 0.78, Ca 8.9, Phos 3.5, Uric acid 3.4, Mg 2.0
Imaging: Non-contrast head CT – no hemorrhage/masses
The patient was seen by ophthalmology and diagnosed with a right retinal hemorrhage. She received follow up information for the retina clinic. The working diagnosis was hypertensive retinopathy. The rest of her exam was unremarkable. Labs were sent given her +ROS for fatigue, headache, nausea and we were soon called about a critical value.
Patient was diagnosed with acute leukemia with concern for blast crisis. In the ED, she was started on IVFs, allopurinol, and hydroxyurea. A peripheral smear showed numerous blasts, no Auer rods. She was admitted to the heme/oncology service for bone marrow biopsy with flow cytometry (showed 84% myeloid blast cells) and induction of chemotherapy.
Take Away #1
Look in the eye! Acute leukemia can present as retinal hemorrhage
One study showed retinal hemorrhages had a prevalence of 42% in a sample of newly diagnosed acute leukemia patients
It usually occurs at the posterior pole of the retina
It can sometimes have a white center (accumulation of leukemic cells)
The eye exam may also reveal cotton wool spots secondary to ischemia from anemia
Take Away #2
Blast crisis: an oncologic emergency
Acute leukemia: 20% or more blasts in the marrow or peripheral blood
Blast crisis (leukostasis): severely elevated blast cell count + signs and symptoms of decreased tissue perfusion
Increased blasts => leukostasis => hyperviscosity => decreased perfusion => ischemia or infarction (MI, CVA, ARF, limb ischemia, bowel infarction, priapism, etc)
20-40% mortality rate in 1 week if left untreated!
Patients usually present in CNS or respiratory distress (hypoxia, headache, AMS, vision changes, stupor, coma)
aggressive hydration with isotonic fluids
usually refrain from using pRBCs unless life-threatening bleeding is present – can increase hyperviscosity
80% are febrile – if there are no localizing symptoms, it is difficult to differentiate infection vs inflammation from leukostasis, so treat empirically.
Keep platelets above 20K to reduce risk of hemorrhage
Heme/onc consult – possible leukapheresis (just a temporizing measure, contraindicated in APL due to worsening coagulopathy) => induction chemotherapy
Take Away #3
Keep differential broad – easy to get tempted by a quick disposition especially in fast track setting, when vitals and exam seem benign and consultant has already recommended outpatient follow up. Do what is best for the patient!
Priyal Patel, MD is a current second year resident at Stony Brook Emergency Medicine.
Edited by Bassam Zahid, MD