جستجوی مقالات مرتبط با کلیدواژه « hemophagocytosis » در نشریات گروه « پزشکی »

Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of clinical syndromes characterized by activation and subsequent uncontrolled non-malignant proliferation of T-lymphocytes, histiocytes and macrophages leading to a cytokine storm and manifesting as prolonged fever, organomegaly, cytopenia, hyperferritinemia and demonstrable hemophagocytosis in the bone marrow.. Here we present a case of infection associated HLH in a five-year-old girl as a complication of Hepatitis A virus infection. The girl developed acute fulminant hepatic failure with multisystem involvement and was treated with a short course of steroid.. Hemophagocytic lymphohistiocytosis is broadly classified as two types, either familial or acquired. The acquired variety may be secondary to any type of infection, mostly viral. Hepatitis A virus is rarely reported to give rise to HLH in the pediatric population. Less intensive immunosuppressive therapy with steroids was successful in maintaining remission..

Macrophage Activation Syndrome (MAS) is an aggressive and potentially life threatening disease which can occur in rheumatologic diseases, more in systemic juvenile idiopathic arthritis and less in adult onset Still’s disease. We presented here a 22-yr-old female who was admitted with high fever for 3 weeks before admission. According to physical and laboratory results and after ruling out infections and malignancies, she was diagnosed with adult onset Still disease.Despite treatment initiation and improvement, she became dramatically ill one week later and jaundice was created. In physical and laboratory data, high fever, pancytopenia and impaired liver function tests and increased serum ferritin levels were found. Bone marrow aspiration and biopsy showed active hemophagocytosis. She was diagnosed with Macrophage Activation Syndrome associated with adult onset Still’s disease and managed with high-dose corticosteroids and cyclosporine. After this treatment she completely recovered.

Hemophagocytic syndrome (HPS) is an uncommon manifestation in systemic lupus erythematosus (SLE). Clinical features of HPS include fever, pancytopenia, abnormal liver enzyme, hepatosplenomegaly, lymphadenopathy, and coagulation disorder. HPS comprises primary and reactive forms. Herein, we describe a case of untreated SLE with HPS as one of the first manifestations of systemic Lupus.

Visceral Leishmaniasis (VL), a systemic infection of the reticuloendotherlial system, is caused by a parasitic infection.The co-occurrence of VL and hemophagocytic syndrome (HPS) has been previously reported in several studies. In this report we present two cases of HPS and VL among members of the same family.