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J Rheum Dis. 2015 Jun;22(3):180-185. Korean. Case Report. https://doi.org/10.4078/jrd.2015.22.3.180
Lim E , Kim YG , Choi WS , Jung YS , Han JH , Bae CB , Jung JY , Kim HA , Suh CH .
Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea,. chsuh@ajou.ac.kr
Department of Laboratory Medicine, Ajou University School of Medicine, Suwon, Korea.
Department of Pathology, Ajou University School of Medicine, Suwon, Korea.
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hyperferritinemia, and phagocytosis of hematopoietic cells in bone marrow, liver, or lymph nodes. HLH can occur during the course of systemic lupus erythematosus (SLE), but can also be a presenting manifestation. Because development of pancytopenia occurs in less than 10 percent of SLE cases, investigation for HLH is necessary when otherwise unexplained pancytopenia persists despite adequate treatment. We experienced three cases of secondary HLH associated with SLE. Among the three patients, two patients developed HLH during the clinical course of SLE. The other patient who presented with pancytopenia was first diagnosed with HLH, and later with SLE. In her case, HLH turned out to be a presenting manifestation of SLE. We report on three successfully treated cases, and discuss the prevalence, characteristics, treatments, and prognosis of secondary HLH associated with SLE.

Copyright © 2019. Korean Association of Medical Journal Editors.