Monocytes From Patients With Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis Are Hyperresponsive to Interferon Gamma

Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?

PURPOSE OF THE REVIEW One of the most perplexing features of systemic-onset juvenile rheumatoid arthritis is the association with macrophage activation syndrome, a life-threatening complication caused by excessive activation and proliferation of T cells and macrophages. The main purpose of the review is to summarize current understanding of the relation between macrophage activation syndrome an...

Death from Hemophagocytic Lymphohistiocytosis Syndrome Due to Generalized Hemorrhage

Hemophagocytic lymphohistiocytosis (HLH) is the result of excessive cytokine release, leading to over-response by immune cells, such as macrophages and T lymphocytes. Here, we report a lethal case of HLH with a complete clinical course. The patient was a 45-year-old man with fever and chills since two months ago plus splenomegaly, hepatomegaly, and pancytopenia. The Anti-HBc IgM was positive, b...

Etoposide in patients with rheuma-associated hemophagocytic lymphohistiocytosis / macrophage activation syndrome

Background/aim Rheuma-associated hemophagocytic lymphohistiocytosis (Rh-HLH), also called macrophage activation syndrome (MAS), is a severe complication of systemic inflammatory disorders. Rh-HLH has clinical and laboratory similarities to other forms of HLH, and is potentially life threatening. Treatment of Rh-HLH has not been standardized yet, but it commonly includes a variety of agents such...

Hemophagocytic lymphohistiocytosis secondary to histoplasmosis

Secondary hemophagocytic lymphohistiocytosis possibly induced by interferon beta-1a therapy.

A 57-year old woman with a history of multiple sclerosis, treated with interferon beta-1a in the last 5 months, was referred for hyperpyrexia (>40°C) that persisted for 15 days. At admission, there was elevation of transaminases, anemia (hemoglobin 8.9 g/dL), thrombocytopenia (platelet 135,000/mm3), and hypofibrinogenemia (fibrinogen 1.26 g/L). C-reactive protein was 10.7 mg/dL, lactate dehy...