Three Cases of Secondary Hemophagocytic Lymphohistiocytosis Associated with Systemic Erythematosus Lupus.
10.4078/jrd.2015.22.3.180
- Author:
Eunsoo LIM
1
;
Young Geon KIM
;
Won Sun CHOI
;
Yu Soek JUNG
;
Jae Ho HAN
;
Chang Bum BAE
;
Ju Yang JUNG
;
Hyoun Ah KIM
;
Chang Hee SUH
Author Information
1. Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea,. chsuh@ajou.ac.kr
- Publication Type:Case Report
- Keywords:
Hemophagocytic lymphohistiocytosis;
Systemic lupus erythematosus;
Pancytopenia
- MeSH:
Bone Marrow;
Fever;
Humans;
Liver;
Lupus Erythematosus, Systemic;
Lymph Nodes;
Lymphohistiocytosis, Hemophagocytic*;
Pancytopenia;
Phagocytosis;
Prevalence;
Prognosis
- From:Journal of Rheumatic Diseases
2015;22(3):180-185
- CountryRepublic of Korea
- Language:Korean
-
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.
