[Skip to Content]
[Skip to Content Landing]

Papilledema in an Adolescent With Anemia and Leukopenia

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 12-year-old girl with an unremarkable medical and ocular history was admitted for workup of anemia (hemoglobin, 6.7 g/dL; to convert to grams per liter, multiply by 10) and leukopenia (white blood cell count, 1220 cells/μL; to convert to ×109 cells per liter, multiply by 0.001) with associated symptoms of headache, malaise, recent cough, and fever. Inflammatory markers were elevated with an erythrocyte sedimentation rate greater than 130 mm/h and a C-reactive protein level of 1.48 mg/dL (to convert to milligrams per liter, multiply by 10). The patient noted floaters in her right eye, which prompted an ophthalmology consultation. On bedside examination, her near visual acuity was J1+ (Snellen equivalent, 20/20 OU), and color vision was full in both eyes; the patient’s intraocular pressure, pupils, extraocular movements, and confrontational visual fields were all unremarkable. Dilated examination revealed grade I and II optic disc edema in the right and left eyes, respectively, and peripapillary hemorrhage in the right eye. Optical coherence tomography was obtained 3 days after initial consultation, which confirmed bilateral disc edema, more pronounced in the left eye than the right eye (Figure 1), and 24-2 Humphrey visual field examination in both eyes was normal. Magnetic resonance imaging (MRI) of the brain and orbits with and without contrast and lumbar puncture were obtained, and results showed diffuse signal abnormality in the bone marrow compartment and findings suggestive of papilledema. Magnetic resonance venography did not reveal thrombosis. Opening pressure during lumbar puncture was 37 cm H2O (upper limit of normal, 28 cm H2O); all cerebrospinal fluid (CSF) viral serology test results were negative, and glucose level and red and white blood cell counts were all within normal limits. CSF protein was slightly decreased at 10.4 mg/dL, and CSF cytospin findings were unremarkable.

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

B-cell acute lymphoblastic leukemia

C. Perform bone marrow biopsy

The patient’s age, markedly elevated inflammatory markers, decreased white and red blood cell counts, signal abnormality within the bone marrow on MRI (Figure 2), and ocular findings raised suspicion for an oncologic process and precluded serial monitoring (choice A) and diagnosis of idiopathic intracranial hypertension (choice B). Acute lymphoblastic leukemia (ALL) and acute myelocytic leukemia were considered as differential diagnoses, as were Hodgkin and non-Hodgkin lymphoma. Of note, idiopathic intracranial hypertension is relatively uncommon in children, with an annual incidence of 0.7 per 100 0001 in patients aged 1 to 16 years, compared with an incidence of 4.7 in 100 000 in adults.2 Serial lumbar punctures would also be inappropriate because the first was nondiagnostic; further, lumbar puncture has a low sensitivity for ALL (choice D).

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 Credit(s)™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

Article Information

Corresponding Author: Aaron Priluck, MD, Wilmer Eye Institute, Johns Hopkins University School of Medicine, 1800 Orleans St, Baltimore, MD 21287 (apriluc1@jh.edu).

Published Online: March 3, 2022. doi:10.1001/jamaophthalmol.2021.5310

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s mother for granting permission to publish this information.

Matthews  YY , Dean  F , Lim  MJ ,  et al.  Pseudotumor cerebri syndrome in childhood.   Arch Dis Child. 2017;102(8):715-721.PubMedGoogle ScholarCrossref
Mollan  SP , Aguiar  M , Evison  F , Frew  E , Sinclair  AJ .  The expanding burden of idiopathic intracranial hypertension.   Eye (Lond). 2019;33(3):478-485.PubMedGoogle ScholarCrossref
Vainionpää  L .  Clinical neurological findings of children with acute lymphoblastic leukaemia at diagnosis and during treatment.   Eur J Pediatr. 1993;152(2):115-119.PubMedGoogle ScholarCrossref
Reddy  SC , Menon  BS .  A prospective study of ocular manifestations in childhood acute leukaemia.   Acta Ophthalmol Scand. 1998;76(6):700-703.PubMedGoogle ScholarCrossref
Bitirgen  G , Belviranli  S , Caliskan  U , Tokgoz  H , Ozkagnici  A , Zengin  N .  Ophthalmic manifestations in recently diagnosed childhood leukemia.   Eur J Ophthalmol. 2016;26(1):88-91.PubMedGoogle ScholarCrossref
Vartzelis  G , Lancaster  D , Fallon  P .  Intracranial hypertension in pediatric patients with acute lymphoblastic leukemia.   Pediatr Blood Cancer. 2009;52(3):418-420.PubMedGoogle ScholarCrossref
Kaikov  Y .  Optic nerve head infiltration in acute leukemia in children.   Med Pediatr Oncol. 1996;26(2):101-104.PubMedGoogle ScholarCrossref
Berk  J , Hall  D , Stroh  I ,  et al.  A child with pancytopenia and optic disc swelling.   Pediatrics. 2019;144(5):e20182887.PubMedGoogle Scholar
Jaime-Pérez  JC , Borrego-López  MF , Jiménez-Castillo  RA ,  et al.  Comparison of conventional cytomorphology, flow cytometry immunophenotyping, and automated cell counting of CSF for detection of CNS involvement in acute lymphoblastic leukemia.   Int J Lab Hematol. 2018;40(2):169-174.PubMedGoogle ScholarCrossref
Kato  M , Manabe  A .  Treatment and biology of pediatric acute lymphoblastic leukemia.   Pediatr Int. 2018;60(1):4-12.PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right

Name Your Search

Save Search
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience

Lookup An Activity


My Saved Searches

You currently have no searches saved.


My Saved Courses

You currently have no courses saved.