Case Report

J Rheum Dis 2015; 22(3): 180-185

Published online June 30, 2015

© Korean College of Rheumatology

전신홍반루푸스에 동반된 이차성 혈구포식성 림프조직구증 3예

임은수1ㆍ김영건1ㆍ최원선1ㆍ정유석2ㆍ한재호3ㆍ배창범1ㆍ정주양1ㆍ김현아1ㆍ서창희1

아주대학교 의과대학 1류마티스내과학교실, 2진단검사의학교실, 3병리학교실

Received: April 1, 2014; Revised: June 26, 2014; Accepted: July 15, 2014

Three Cases of Secondary Hemophagocytic Lymphohistiocytosis Associated with Systemic Erythematosus Lupus

Eunsoo Lim1, Young-Geon Kim1, Won-Sun Choi1, Yu-Soek Jung2, Jae-Ho Han3, Chang-Bum Bae1, Ju-Yang Jung1, Hyoun-Ah Kim1, Chang-Hee Suh1

Departments of 1Rheumatology, 2Laboratory Medicine, and 3Pathology, Ajou University School of Medicine, Suwon, Korea

Correspondence to : Chang-Hee Suh

Received: April 1, 2014; Revised: June 26, 2014; Accepted: July 15, 2014

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 devel-oped 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. (J Rheum Dis 2015;22:180-185)

Keywords Hemophagocytic lymphohistiocytosis, Systemic lupus erythematosus, Pancytopenia

Article

Case Report

J Rheum Dis 2015; 22(3): 180-185

Published online June 30, 2015

Copyright © Korean College of Rheumatology.

전신홍반루푸스에 동반된 이차성 혈구포식성 림프조직구증 3예

임은수1ㆍ김영건1ㆍ최원선1ㆍ정유석2ㆍ한재호3ㆍ배창범1ㆍ정주양1ㆍ김현아1ㆍ서창희1

아주대학교 의과대학 1류마티스내과학교실, 2진단검사의학교실, 3병리학교실

Received: April 1, 2014; Revised: June 26, 2014; Accepted: July 15, 2014

Three Cases of Secondary Hemophagocytic Lymphohistiocytosis Associated with Systemic Erythematosus Lupus

Eunsoo Lim1, Young-Geon Kim1, Won-Sun Choi1, Yu-Soek Jung2, Jae-Ho Han3, Chang-Bum Bae1, Ju-Yang Jung1, Hyoun-Ah Kim1, Chang-Hee Suh1

Departments of 1Rheumatology, 2Laboratory Medicine, and 3Pathology, Ajou University School of Medicine, Suwon, Korea

Correspondence to:Chang-Hee Suh

Received: April 1, 2014; Revised: June 26, 2014; Accepted: July 15, 2014

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 devel-oped 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. (J Rheum Dis 2015;22:180-185)

Keywords: Hemophagocytic lymphohistiocytosis, Systemic lupus erythematosus, Pancytopenia

JRD
Jan 01, 2024 Vol.31 No.1, pp. 1~63
COVER PICTURE
Characteristic findings in interstitial lung disease (ILD) detected by lung ultrasound. Lung ultrasound can reveal characteristic findings in ILD. However, the definitive diagnosis of ILD typically requires a combination of clinical assessment, imaging studies (such as high-resuloution computed tomography [HRCT]), and sometimes a lung biopsy. (A) Traction bronchiectasis and parenchymal changes of upper lung in HRCT (arrows). (B) Corresponding changes of lung ultrasound presented by B-lines (arrows). (J Rheum Dis 2024;31:3-14)

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