Familial hemophagocytic lymphohistiocytosis (HLH) is a rare primary immunodeficiency disorder characterized by defects in cell-mediated cytotoxicity that results in fever, hepatosplenomegaly, and cytopenias. Familial HLH is well recognized in children but rarely diagnosed in adults. We conducted a retrospective review of genetic and immunologic test results in patients who developed HLH in adul...

Primary Epstein-Barr virus (EBV) infection causes infectious mononucleosis and hemophagocytic lymphohistiocytosis (HLH) in children, where EBV infects B and CD8(+) T cells, respectively. We measured pro-inflammatory and anti-inflammatory cytokines in both diseases. Significantly higher concentrations of various mediators, including interferon-γ, neopterin, interleukin (IL)-6, IL-10, IL-18, and ...

Hemophagocytic lymphohistiocytosis (HLH), which is associated with various underlying conditions, is characterized by hypercytokinemia. Because it is frequently lethal, immediate mitigation of the hypercytokinemia is vital to save patients, particularly when treatments for the patient's underlying condition are ineffective on HLH. We herein present a case of Hodgkin lymphoma associated with HLH...

A 16-year-old boy with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (HLH) underwent allogeneic hematopoietic stem cell transplantation after conditioning with fludarabine, melphalan, total body irradiation, and rabbit antithymocyte globulin (ATG). A severe, persistent infusion reaction occurred after the initial administration of ATG. Investigations showed a rapid increase i...

Hemophagocytic lymphohistiocytosis (HLH) is a rare and devastating disorder of uncontrolled immune activation that has gained increasing recognition over the past decade. Patients with HLH present with clinical and laboratory evidence of extreme inflammation. This syndrome was first described in 1939 by Scott and Robb-Smith and again in 1952 when Farquhar and Claireaux reported a case of 2 infa...

Genetic disorders of lymphocyte cytotoxicity predispose patients to hemophagocytic lymphohistiocytosis (HLH). Reduced lymphocyte cytotoxicity has been demonstrated in Hermansky-Pudlak syndrome type 2 (HPS2), but only a single patient was reported who developed HLH. Because that patient also carried a potentially contributing heterozygous RAB27A mutation, the risk for HLH in HPS2 remains unclear...

Metabolic clearance rates and production rates of human luteinizing hormone (HLH) were determined in pre- and postmenopausal women by the constant infusion technique. Highly purified HLH-(131)I was infused into the fasting subjects at a constant rate. Serial plasma samples were obtained and the radioactive hormone was precipitated by a double antibody technique. Plasma HLH-(131)I levels reached...

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare multiorgan disease of toxic immune activation caused by the interaction of cytotoxic T cells and innate immune cells and frequently involves the central nervous system (CNS). Posterior reversible encephalopathy syndrome (PRES) might develop during treatment with the HLH-2004 protocol from the Histiocyte Society. The aims of this stud...

This paper deals with the question whether automatic and non-automatic downstep are distinct processes or can be regarded as the same phenomenon. Downstep is seen as a lowering of the pitch register, relatively to which tones are scaled. The distinction between the two types of downstep arises from differences in their tonal surface structure: In the case of automatic downstep a L(ow) tone inte...