[Generalized angitis and recurrent infection associated hemophagocytic syndrome in a girl with XXX syndrome].
We have encountered a case of a girl with XXX syndrome who had infection associated hemophagocytic syndrome (HPS) recurrently. The patient presented hyper-gammaglobulinemia during the clinical course and developed IAHS probably because of infection with Rubella virus and EB virus each as a trigger. Diseases that cause abnormality in X chromosome are said to present immune abnormality such as SLE in many cases. It is possible that the excessive X chromosome in this cases partially concerned with such as an immune abnormality as to cause recurrent IAHS.
Wada
Y
Y
Department of Pediatrics, Kashiwa Hospital, Tokyo Jikei University School of Medicine, Chiba.
Satoh
T
T
Kubo
M
M
D000293:Adolescent; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology; D007154:Immune System Diseases / Q000150:complications; D007244:Infectious Mononucleosis; D012008:Recurrence; D012409:Rubella; D012729:Sex Chromosome Aberrations; D014657:Vasculitis / Q000209:etiology*; D014960:X Chromosome