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Hemophagocytic Lymphohistiocytosis After Dengue Fever and Its Ocular Features

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1 Credit CME

A man in his early 40s presented with history of dengue fever requiring admission in the intensive care unit. His serum inflammatory parameters were markedly elevated: lactate dehydrogenase, 939 IU/L (normal, 100-250 IU/L; to convert to microkatals per liter, multiply by 0.0167); ferritin, 16 282 ng/mL (normal, 30-400 ng/mL; to convert to micrograms per liter, multiply by 1); C-reactive protein, 101 mg/L (normal, <5 mg/L; to convert to milligrams per deciliter, divide by 10); triglycerides, 667 mg/dL (normal, <150 mg/dL; to convert to millimoles per liter, multiply by 0.0113); and serum fibrinogen, 363 mg/dL (normal, 180-350 mg/dL; to convert to grams per liter, multiply by 0.01). The patient had developed thrombocytopenia, anemia, hyponatremia, viral meningoencephalitis, transaminitis, splenomegaly, and acute kidney injury. He was diagnosed with hemophagocytic lymphohistiocytosis (HLH)1 secondary to dengue fever. We reported the ophthalmic features in this case of HLH. Visual acuity was 20/20 OU, anterior-segment examination was normal, and fundus examination revealed vitritis, 1+ vitreous haze, nerve fiber layer infarcts, retinal hemorrhages, and whitish outer retinal lesions in the inferior periphery in both eyes (Figure, A). Optical coherence tomography over the whitish lesions revealed subretinal pigment epithelium deposits, which could be the lymphohistiocytic aggregates seen in HLH as described in the literature (Figure, B).2 At a recent follow-up visit, visual acuity was stable with resolving fundus lesions.

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Article Information

Corresponding Author: Padmamalini Mahendradas, DNB, Department of Uveitis and Ocular Immunology, Narayana Nethralaya Eye Institute, 121/C, Chord Road, Rajajinagar, 1st ‘R’ Block, Bangalore 560010, India (m.padmamalini@gmail.com).

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information and Mallikarjun Kalashetty, DM (Manipal Hospital, Bangalore, India), for managing the systemic condition of the patient. No one received financial compensation for their contribution.

References
1.
Henter  JI , Horne  A , Aricó  M ,  et al.  HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.   Pediatr Blood Cancer. 2007;48(2):124-131. doi:10.1002/pbc.21039PubMedGoogle ScholarCrossref
2.
Vizcaino  MA , Eberhart  CG , Rodriguez  FJ .  Hemophagocytic lymphohistiocytosis in adults with intraocular involvement: clinicopathologic features of 3 cases.   Ocul Oncol Pathol. 2017;4(1):1-11. doi:10.1159/000475551PubMedGoogle 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 credit toward the CME of the American Board of Surgery’s Continuous Certification program

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

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