A 66-year-old man presented with decompensated acute liver failure with no clear etiology. Eventually, a bone marrow biopsy revealed hemophagocytic cells, and he was diagnosed with hemophagocytic lymphohistiocytosis. This is an uncommon condition in adults, with high morbidity and mortality, and it is often indistinguishable from other forms of acute liver failure. Early clinical suspicion is c...

Infection-associated hemophagocytic syndrome is a life-threatening condition characterized by prolonged fever, hepatosplenomegaly, and cytopenia-most commonly, thrombocytopenia and anemia. It is characterized by proliferation and activation of benign histiocytes, causing dysfunction of various organs. Herein, we report on a 5-month-old boy whose clinical picture and laboratory findings were con...

INTRODUCTION Hemophagocytic lymphohistiocytosis induced by viral diseases is a well recognized entity. Severe forms of H5N1 influenza are known to be associated with symptoms very similar to a reactive hemophagocytic syndrome. We report a case of fulminant lymphohistiocytosis associated with the pandemic A (H1N1) variant. CASE PRESENTATION A 42-year-old Caucasian woman developed a syndrome of...

Wolman disease; Familial hemophagocytic lymphohistiocytosis; Hepatomegaly; Splenomegaly; Fever Abstract Background: Familial hemophagocytic lymphohistiocytosis is a rare autosomal recessive disease that is usually evident in the first few months or years of life. Major signs and symptoms include hepatomegaly, splenomegaly, anemia, leucopenia or thrombocytopenias which resemble many inborn error...

OBJECTIVE To investigate the frequency of hemophagocytic syndrome in a series of patients with otherwise unexplained cytopenia. MATERIAL AND METHOD In this cross-sectional, single-centre study, bone marrow specimens (n=288) were obtained from the patients with unexplained cytopenia. The diagnosis of hemophagocytic syndrome was made according to universally accepted criteria. Characteristics o...

BACKGROUND Total colonic and small bowel aganglionosis is a rare condition typically requiring intestinal transplant for long-term survival. There have not been any previously reported cases of near total intestinal aganglionosis complicated by concerns for hemophagocytic lymphohistiocytosis and need for both multivisceral organ transplant and hematopoietic stem cell transplant. CASE PRESENTA...

Salmonella enterica subspecies can establish persistent, systemic infections in mammals, including human typhoid fever. Persistent S. enterica disease is characterized by an initial acute infection that develops into an asymptomatic chronic infection. During both the acute and persistent stages, the bacteria generally reside within professional phagocytes, usually macrophages. It is unclear how...

BACKGROUND Adult hemophagocytic lymphohistiocytosis is a secondary immunopathologic phenomenon, mainly secondary to malignancy, infection, or autoimmune disorders. The performance of diagnostic criteria, studied in the pediatric population, is yet to be validated in the adult population. Some of the criteria include cytopenias and organomegaly that are inherent features to malignant processes, ...

conclusions the occurrence of sam in pauci-autoimmune vasculitis is rarely described, particularly in africa. our case is an illustration of the reality of this association. introduction we reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis. case presentation a 22-year-old female patient originated from dakar, senegal prese...