Abstract
A 26-year-old woman presented with general fatigue, persistent fever, nuchal lymphadenitis, thrombocytopenia, and liver damage. From the bone marrow finding, we diagnosed her condition as hemophagocytic syndrome. Steroid pulse therapy, cyclosporin A treatment, and combined chemotherapy generated no response. The patient showed severe mucosal bleeding, rapidly experienced multiple organ failure, and finally died of a brain hemorrhage on the 13th hospital day. Epstein-Barr virus, cytomegalovirus, human herpes virus type 6, human parvovirus B19, and herpes simplex virus were not detected. Autopsied samples of the spleen, bone marrow, and liver showed extreme proliferation of activated macrophages, so-called histiocytes, without lymphoid malignancy. The interferon gamma level at presentation was prominently high. The continuously elevated levels of ferritin and soluble interleukin 2 receptor were correlated with the catastrophic outcome. The disease in our case mimicked infantile hemophagocytic lymphohistiocytosis. However, there was neither a family history of the disease nor a mutation in the perforin gene. So, it is reasonable to categorize our case as macrophage activation syndrome. Although our patient lacked arthritis or erup...Continue Reading
References
Sep 1, 1979·Cancer·R J RisdallR D Brunning
Jul 7, 1990·Lancet·L M HuangD T Lin
Mar 1, 1991·British Journal of Haematology·S Imashuku, S Hibi
Jan 1, 1989·Pediatric Hematology and Oncology·D M KompJ van Hoff
Mar 1, 1989·Acta paediatrica Scandinavica·N EsumiS Imashuku
May 15, 1988·Cancer·N EsumiS Imashuku
Jun 1, 1980·The American Journal of Medicine·J M EsdaileD Hawkins
Jun 1, 1993·Journal of Hepatology·M LasserreO Terno
Jun 1, 1996·British Journal of Haematology·H Tsuda, K Shirono
Nov 1, 1996·The Journal of Pediatrics·A A Grom, M Passo
Apr 1, 1997·International Journal of Hematology·H Tsuda
Jul 28, 2001·Annals of Hematology·Y TakeokaN Tatsumi
Sep 18, 2001·American Journal of Hematology·U EmmeneggerK A Neftel
Sep 17, 2002·Blood·Rita ClementiMaurizio Aricó
Mar 18, 2003·The Journal of Pediatrics·Alexei A GromAlexandra Filipovich
Apr 29, 2003·British Journal of Haematology·Ikuyo UedaShinsaku Imashuku
May 13, 2003·Clinical Rheumatology·A MertY Aktuglu
Jun 26, 2003·Medical and Pediatric Oncology·Shinsaku ImashukuShigeyoshi Hibi
Sep 10, 2003·Current Opinion in Rheumatology·Alexei A Grom
Citations
Apr 25, 2006·Modern Rheumatology·Rie TabataHiroyuki Kotani
May 12, 2010·Journal of Microbiology, Immunology, and Infection = Wei Mian Yu Gan Ran Za Zhi·Erol ErduranMehmet Mutlu
Dec 27, 2005·Clinics in Liver Disease·John BucuvalasRobert H Squires
Feb 1, 2007·Arthritis and Rheumatism·Donato RiganteAchille Stabile
Jan 15, 2008·International Journal of Hematology·Anastasios AndreopoulosGeorge Vaiopoulos
Jan 30, 2021·World Journal of Clinical Cases·Fan YuJiang-Bin Wang