PMID- 27826329
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200930
IS  - 1738-1061 (Print)
IS  - 2092-7258 (Electronic)
IS  - 1738-1061 (Linking)
VI  - 59
IP  - 10
DP  - 2016 Oct
TI  - Recurrent macrophage activation syndrome since toddler age in an adolescent boy 
      with HLA B27 positive juvenile ankylosing spondylitis.
PG  - 421-424
AB  - Recurrent macrophage activation syndrome (MAS) is very rare. We present the case 
      of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing 
      spondylitis (AS), who experienced episodes of recurrent MAS since he was a 
      toddler. A 16-year-old boy was admitted because of remittent fever with 
      pancytopenia and splenomegaly after surgical intervention for an intractable 
      perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis 
      (HLH) 4 different times, which was well controlled with intravenous 
      immunoglobulin and steroids since the age of 3. We were unable to identify the 
      cause for the HLH. He remained symptom-free until the development of back pain 
      and right ankle joint pain with swelling at 15 years of age. He was diagnosed 
      with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom 
      aggravation despite taking naproxen and methotrexate, and the symptoms improved 
      with etanercept. On admission, his laboratory data showed leukopenia with high 
      ferritin and triglyceride levels. Bone marrow biopsy examination showed 
      histiocytic hyperplasia with hemophagocytosis. There was no evidence of 
      infection. He received naproxen alone, and his symptoms and laboratory data 
      improved without any other immunomodulatory medications. Genetic study revealed 
      no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune 
      disease should have been considered as the cause of recurrent MAS in the young 
      patient once primary HLH was excluded.
FAU - Park, Joon Hyeong
AU  - Park JH
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.
FAU - Seo, Yu Mi
AU  - Seo YM
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.
FAU - Han, Seung Beom
AU  - Han SB
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.; Vaccine Bio Research Institute, College of Medicine, The Catholic 
      University of Korea, Seoul, Korea.
FAU - Kim, Ki Hwan
AU  - Kim KH
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.
FAU - Rhim, Jung Woo
AU  - Rhim JW
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.
FAU - Chung, Nack Gyun
AU  - Chung NG
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.
FAU - Kim, Myung Shin
AU  - Kim MS
AD  - Department of Laboratory Medicine, Catholic Genetic Laboratory Center, Seoul St. 
      Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, 
      Korea.
FAU - Kang, Jin Han
AU  - Kang JH
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.; Vaccine Bio Research Institute, College of Medicine, The Catholic 
      University of Korea, Seoul, Korea.
FAU - Jeong, Dae Chul
AU  - Jeong DC
AD  - Department of Pediatrics, College of Medicine, The Catholic University of Korea, 
      Seoul, Korea.; Vaccine Bio Research Institute, College of Medicine, The Catholic 
      University of Korea, Seoul, Korea.
LA  - eng
PT  - Journal Article
DEP - 20161017
PL  - Korea (South)
TA  - Korean J Pediatr
JT  - Korean journal of pediatrics
JID - 101215374
PMC - PMC5099290
OTO - NOTNLM
OT  - Ankylosing spondylitis
OT  - HLA-B27
OT  - Macrophage activation syndrome
COIS- No potential conflict of interest relevant to this article was reported.
EDAT- 2016/11/09 06:00
MHDA- 2016/11/09 06:01
PMCR- 2016/10/01
CRDT- 2016/11/10 06:00
PHST- 2016/05/03 00:00 [received]
PHST- 2016/08/08 00:00 [revised]
PHST- 2016/08/18 00:00 [accepted]
PHST- 2016/11/10 06:00 [entrez]
PHST- 2016/11/09 06:00 [pubmed]
PHST- 2016/11/09 06:01 [medline]
PHST- 2016/10/01 00:00 [pmc-release]
AID - 10.3345/kjp.2016.59.10.421 [doi]
PST - ppublish
SO  - Korean J Pediatr. 2016 Oct;59(10):421-424. doi: 10.3345/kjp.2016.59.10.421. Epub 
      2016 Oct 17.