Reactive Hemophagocytic syndrome
Publication Type:Journal Article
Source:Indian Journal of Pediatrics, Volume 75, Number 12, p.1261-1263 (2008)
Keywords:adolescent, antibiotic agent, arthralgia, Arthritis, article, blood clotting disorder, Blood Coagulation Disorders, bone marrow biopsy, Cardiac Tamponade, case report, childhood disease, chill, cyanosis, disease severity, drug, dyspnea, erythrophagocytosis, female, fever, fresh frozen plasma, Glucocorticoids, heart tamponade, Hemophagocytic, hemophagocytic syndrome, hepatomegaly, human, Humans, hydroxychloroquine, indometacin, Juvenile Rheumatoid, juvenile rheumatoid arthritis, laboratory test, liver, liver dysfunction, Lupus Erythematosus, Lymphohistiocytosis, methylprednisolone, naproxen, pericardial effusion, physical examination, prednisolone, pulsatile drug release, Pulse Therapy, reactive hemophagocytic syndrome, Sepsis, shock, splenomegaly, symptomatology, Systemic, systemic disease, systemic lupus erythematosus, thorax pain, thorax radiography
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. © 2008 Dr. K C Chaudhuri Foundation.
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