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A Rare Case of Hemophagocytic Lymphohistiocytosis Associated With Myelodysplastic Syndrome and Trisomy 8 in a Pediatric Patient

Kim, Seh Hyun, MD; Yi, Dae Yong, MD; Lee, Na Mi, MD; Yun, Sin Weon, MD; Chae, Soo Ahn, MD; Lim, In Seok, MD

Journal of Pediatric Hematology/Oncology: January 2019 - Volume 41 - Issue 1 - p e57–e59
doi: 10.1097/MPH.0000000000001174
Online Articles: Clinical and Laboratory Observations

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting in clinical and biochemical manifestations of extreme inflammation. Myelodysplastic syndrome (MDS) represents a heterogenous group of clonal hematopoietic disorders. The development of MDS is common in children with trisomy of chromosome 8. Here, we report a fatal case of 8-year-old girl who was admitted to the emergency department with status epilepticus, and later diagnosed with HLH associated with MDS and trisomy of chromosome 8. We believe this is the first reported case of HLH associated with MDS and trisomy 8 in a pediatric patient.

Department of Pediatrics, Chung-Ang University College of Medicine, Seoul, Korea

The authors declare no conflict of interest.

Reprints: Soo Ahn Chae, MD, Department of Pediatrics, Chung-Ang University Hospital, 102, Heukseok-ro, Dongjak-gu, Seoul 06973, Korea (e-mail:

Received June 27, 2017

Accepted February 28, 2018

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