Reactive hemophagocytic syndrome.
We describe two cases of Reactive Hemophagocytic syndrome (RHS) occurring in rheumatic diseases in childhood. Patient 1, an adolescent girl with systemic onset Juvenile idopathic arthritis (JRA) presented like severe sepsis with shock, hepatic dysfunction and coagulopathy. Patient 2 presented with cardiac tamponade, she was later detected to have systemic lupus erythematosus (SLE). Her bone marrow aspirate revealed prominent hemophagocytosis. Both cases improved with pulse methylprednisolone therapy.
Rajam
L
L
Department of Pediatrics, Amrita Institute of Medical Sciences and Research Center, Kochi, Kerala, India.
Prasad
Vinitha
V
Yatheesha
B L
BL
D000293:Adolescent; D001171:Arthritis, Juvenile / Q000150:complications*; D001778:Blood Coagulation Disorders / Q000209:etiology; D002305:Cardiac Tamponade / Q000209:etiology; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D008099:Liver / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008775:Methylprednisolone / Q000008:administration & dosage; D020551:Pulse Therapy, Drug
D005938:Glucocorticoids; D008775:Methylprednisolone