histiocytic lesion

(redirected from Reactive hemophagocytic syndrome)

histiocytic lesion

An aggregate of histiocytes, which may be benign, indeterminant, or malignant in proliferative potential; to reduce confusion, it has been suggested that histiocytoses be subdivided into different categories
Histiocytic lesions-Behavior classification  
Benign
Familial histiocytosis with eosinophilia A chronic disease of infants with recurring bacterial infections, diarrhea, eczema, alopecia, associated with immunodeficiency
Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman disease A disease most common in adolescent blacks with massive cervical lymphadenopathy as well as enlargement of extranodal (orbit, skin, bone, salivary gland, testis) lymphoid tissues
Virus-associated hemophagocytic syndrome A condition induced by viral infections, often accompanied by abnormal liver function tests, coagulation assays and pancytopenia Pathology Histiocyte hyperplasia, hemophagocytosis and replacement of native bone marrow elements
Intermediate
Histiocytosis X, aka Langerhans' cell histiocytosis, see there.
Reactive hemophagocytic syndrome, see there.
Malignant
Histiocytic medullary reticulosis see there.
Histiocytic proliferations, eg—acute monocytic leukemia (FAB M3), histiocytic lymphoma see there, malignant histiocytosis–see there.
References in periodicals archive ?
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.