Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disorder, characterized by a hyperimmune response. The mortality is high despite progress being made in the diagnosis and treatment of the disease. HLH is traditionally divided into primary (familial or genetic) and secondary (reactive) according to the etiology. Secondary HLH (sHLH), more common in adults, is often associated with underlying conditions including severe infections, malignancies, autoimmune diseases, or other etiologies. The case involves a 31-year-old woman, presented with a high persistent fever, rash, and splenomegaly. She met the diagnostic criteria of the HLH-2004 guideline and thus was diagnosed with HLH, with positive anti-nuclear antibody (ANA) and positive cytomegalovirus (CMV)-DNA. The patient responded well to a combination of immunomodulatory, chemotherapy, and supportive treatments. When her PCR evaluation for CMV turned negative, her serum ferritin also dropped significantly. Her clinical symptoms improved dramatically, and except for ANA, the abnormal laboratory findings associated with HLH returned to normal. Our previous study has shown that the median overall survival of HLH patients is only 6 mo; however, our patient has b...Continue Reading
References
Feb 1, 1987·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·F M BalisD G Poplack
Feb 1, 1995·British Journal of Haematology·R L ChenC Y Lee
Feb 16, 2000·Leukemia & Lymphoma·S ImashukuM Konno
Mar 1, 2002·Internal Medicine·Osamu SakamotoMasayuki Ando
May 19, 2004·Diseases of the Colon and Rectum·Shin-Ichiro KoketsuHirokazu Nagawa
Jun 2, 2006·Journal of Paediatrics and Child Health·Winita HardikarRichard Couper
Aug 29, 2006·Pediatric Blood & Cancer·Jan-Inge HenterGritta Janka
Sep 13, 2007·American Journal of Hematology·Arnaud HotJacques Ninet
Dec 19, 2007·Pediatric Blood & Cancer·Carl E AllenKenneth L McClain
Sep 11, 2008·Rheumatology·S FukayaT Koike
Aug 11, 2011·Blood·Michael B JordanKenneth L McClain
Sep 24, 2011·Journal of Crohn's & Colitis·Daniel R van LangenbergPeter R Gibson
Feb 11, 2012·Journal of Pediatric Hematology/oncology·Juli Renate SwitalaAlan Davidson
Dec 3, 2013·Lancet·Manuel Ramos-CasalsXavier Bosch
Apr 24, 2014·Arthritis & Rheumatology·Shunichi Kumakura, Yohko Murakawa
Jun 4, 2014·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·Dennis W SimonE Scott Halstead
Sep 24, 2014·Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi·Wenqiu HuangZhao Wang
Jan 13, 2015·Blood·Alison M SchramNancy Berliner
Mar 12, 2015·Blood·Alison M Schram, Nancy Berliner
Sep 25, 2017·Blood·Elisabet BergstenJan-Inge Henter
Jan 25, 2018·Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi·X GaoT Tian
Apr 18, 2019·Blood·Paul La RoséeJan-Inge Henter
Jul 13, 2019·Blood·Matt S Zinter, Michelle L Hermiston
Feb 29, 2020·Blood·Scott W Canna, Rebecca A Marsh
Mar 14, 2020·Orphanet Journal of Rare Diseases·Jun ZhouHua-Guo Xu