Familial hemophagocytic lymphohistiocytosis (FHL) is a potentially lethal genetic disorder of immune dysregulation that requires prompt and accurate diagnosis to initiate life-saving immunosuppressive therapy and to prepare for hematopoietic stem cell transplantation. In the present study, 85 patients with hemophagocytic lymphohistiocytosis were screened for FHL3 by Western blotting using plate...

Familial hemophagocytic lymphohistiocytosis (FHL) is an inherited, fatal disorder of infancy. We report here a 17-day-old female infant who presented with high fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, thrombocytopenia, and liver failure. Leukocytosis was detected with circulating "atypical" lymphoid cells. Flow cytometric studies revealed expanded subpopulations of C...

EBV associated hemophagocytic lymphohistiocytosis and EBV-positive T cell lymphoproliferative disease of childhood share many histologic and clinical features, which sometimes makes it very difficult to render a definitive diagnosis. In this report, we present a 16-year-old male who developed symptoms clinically consistent with EBV associated hematophagocytic lymphohistiocytosis including fulfi...

Haemophagocytic lymphohistiocytosis (HLH) describes a clinical syndrome of hyperinflammation resulting in an uncontrolled and ineffective immune response. It may develop subsequent to a number of recognised genetic mutations or in association with infection, malignancy, autoinflammatory or metabolic conditions. Even with the published diagnostic criteria it can be difficult to make the diagnosi...

A 34-year-old male with a history of T cell–rich B-cell lymphoma relapsed 1 year after high-dose therapy with recurrent fevers, abdominal pain, and jaundice. He was cachetic with hepatosplenomegaly and ascites. Laboratory findings showed a white blood cell count of 2.7 109/L, hemoglobin 8.7 g/dL, platelets of 21 109/L, hyperbilirubinemia, and no evidence of hemolysis including a negative Coombs...

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, HLH is an acquired syndrome. We report a case of a nine month-old-boy presented with hepatosplenomegaly, severe anemia, thrombocytopenia, hypertriglyceridemia and high hyperferritinemia. These clinical features of HLH prompted a w...

Dear Sir, I read with interest a recent article by Antonodimitrakis and colleagues in your journal presenting a case of acquired hemophagocytic lymphohistiocytosis (HLH) in a 60-year-old woman suffering from diabetes mellitus type 2 (1). The reported patient developed a life-threatening HLH associated with a reactivation of an Epstein–Barr virus (EBV) infection and was successfully treated by m...

Corresponding author: Jeongmin Kim, M.D., Department of Anesthesiology and Pain Medicine, Anesthesia and Pain Research Institute, Yonsei University College of Medicine, 50, Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea. Tel: 82-2-2227-3569, Fax: 82-2-2227-7897, E-mail: [email protected] This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commerc...

Graft-versus-host disease (GVHD) is a common complication of bone marrow transplantation (BMT) that can be classified as acute or chronic. Chronic GVHD, which usually occurs more than 3 months after BMT, includes typical lichenoid or sclerodermatous lesions. Psoriasiform eruption is a rare clinical manifestation of chronic GVHD, and there have been no reports of psoriasiform chronic GVHD associ...