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Reactive hemophagocytic syndrome is clinically characterized by fever, hepatosplenomegaly, pancytopenia, and coagulopathy, and is histologically characterized by excessive proliferation and activation of histiocytes or macrophages.
This diversity has led to the suggestion that HLH secondary to an underlying medical illness should be regarded as reactive hemophagocytic syndrome.
High dose gamma-globulin therapy in the reactive hemophagocytic syndrome.
Abstract: Reactive hemophagocytic syndrome (RHS) is an uncommon life-threatening disorder.
Key Words: autopsy, fungal endocarditis, disseminated histoplasmosis, reactive hemophagocytic syndrome
Reactive hemophagocytic syndrome (RHS) is a potentially fatal disorder characterized by marked activation of the monocyte-macrophage cell line leading to excessive hemophagocytosis.
2,8] Cheng et al[8] reported a case of IVL presenting with encephalomyelitis and reactive hemophagocytic syndrome diagnosed by renal biopsy.
Intravascular lymphomatosis: a case presenting with encephalomyelitis and reactive hemophagocytic syndrome diagnosed by renal biopsy.
This diversity has prompted the suggestion that HLH secondary to an underlying medical illness should be designated reactive hemophagocytic syndrome.
Subsequently, HLH has been reported in association with a variety of infections, and the term reactive hemophagocytic syndrome has been suggested to distinguish HLH associated with an identifiable infectious or noninfectious etiology from its hereditary forms.
Successful treatment of reactive hemophagocytic syndrome with cyclosporin A and intravenous immunoglobulin.