INTRODUCTION Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, cytopenias, hepatosplenomegaly and hemophagocytosis. HLH may be primary or secondary to infection, autoimmune disease or malignancy. Hypertriglyceridemia is a common abnormality in HLH and one of the HLH-2004 diagnostic criteria. CASE OUTLINE We present an infant with severe h...

Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that can be associated with inherited genetic mutations, malignancy, autoimmune disorders, and viral infections. Though the pathogenesis is not fully known, HLH is understood to be a reactive process in the setting of uncontrolled activation of macrophages, CD8+ cytotoxic lymphocytes, and other immune ce...

We report a case of a patient who presented with hemophagocytic syndrome (HPS) and adrenal crisis associated with bilateral adrenal gland tuberculosis, and resulted in a poor outcome. A 50-year-old man was transferred to our hospital from a local clinic due to fever, weight loss, and bilateral adrenal masses. Laboratory findings showed leukopenia, mild anemia, and elevated lactate dehydrogenase...

which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. A 1 5/12-year-old girl was admitted with persistent high fever accompanied by mild cough. Physical examination revealed moderate hepatomegaly and splenomegaly. Laboratory tests revealed bicytopenia (hemoglobin level, 10.5 g/dL; white blood cell count, 2.9×10...

Dengue is endemic in more than 100 countries in Southeast Asia, the Americas, the western Pacific, Africa and the eastern Mediterranean regions. The virus is transmitted by Aedes mosquitoes. Dengue disease is the most prevalent arthropod-borne viral disease in humans and is a global and national public health concern in several countries. A seasonal pattern of dengue disease is consistently obs...

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory/hypercytokinemia syndrome clinicopathologically manifested by fever, hepatosplenomegaly, cytopenias, liver dysfunction, and hemophagocytosis. METHODS We searched the medical literature for English-written articles and analyzed data regarding the diagnosis, pathoetiology, prognosis, and managem...

Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic condition characterized by high fever, hepatosplenomegaly, cytopenia, hyperferritinemia, and increased hemophagocytic macrophage proliferation and activation in the reticuloendothelial system. Primary HLH is familial and is a fatal disease that begins during early childhood. Secondary HLH may be acquired after intense activation of...

Autoinflammatory diseases are caused by pathologic activation of the innate immune system. Primary hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation caused by monogenic mutations resulting in cytotoxic cell defects and subsequent failure to eliminate activated macrophages. Secondary HLH is often diagnosed in cases without a known Mendelian inherit...

within a given pedigree can only be explained by a ‘‘second signal.’’ In our patient the nature of this second signal was shown not to be one of the known genes involved in hemophagocytosis. Once intravenous immune globulin and prednisone were started, ferritin and sIL-2R levels did not completely normalize (ferritin, 600-750 mg/L [upper limit of normal, 350 mg/L]; sIL-2R, 4320 U/mL [upper limi...