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EBV-related Cold Agglutinin Disease Presenting With Conjugated Hyperbilirubinemia

A Pediatric Case Report and Mini Review

Mantadakis, Elpis, MD, PhD*; Chatzimichael, Eleftherios, MS; Kontekaki, Eftychia, MD; Panopoulou, Maria, MD, PhD§; Martinis, Georges, MD; Tsalkidis, Aggelos, MD, PhD*

Journal of Pediatric Hematology/Oncology: May 2019 - Volume 41 - Issue 4 - p 324–327
doi: 10.1097/MPH.0000000000001184
Clinical and Laboratory Observations

Hemolytic anemia occurs in only 1% to 3% of hospitalized patients with infectious mononucleosis. The authors describe an 8-year-old girl without cervical lymphadenopathy or splenomegaly, who presented with conjugated hyperbilirubinemia and was diagnosed with cold agglutinin disease caused by an immunoglobulin M autoantibody with anti-i specificity. Acute Epstein-Barr virus infection was confirmed by serologic and molecular methods. She recovered uneventfully after a 3-week course of methylprednisolone. Epstein-Barr virus infection should be considered in any case of hemolytic anemia associated with hepatic dysfunction, especially when direct antiglobulin test is positive for C3d. In these cases, a course of corticosteroids seems safe and may be beneficial.

Departments of *Pediatrics

§Microbiology, Democritus University of Thrace Faculty of Medicine

Democritus University of Thrace Medical School

Blood Transfusion Center, University General Hospital of Alexandroupolis, Alexandroupolis, Thrace, Greece

The authors declare no conflict of interest.

Reprints: Elpis Mantadakis, MD, PhD, Department of Pediatrics, Democritus University of Thrace Faculty of Medicine, University General Hospital of Alexandroupolis, 6th Kilometer Alexandroupolis-Makris, Alexandroupolis 68100, Thrace, Greece (e-mail:

Received December 12, 2017

Accepted March 23, 2018

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