This paper presents to the surgical community an unusual and often ignored cause of gastrointestinal bleeding. Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a rare medical entity characterized by phagocytosis of red blood cells, leucocytes, platelets, and their precursors in the bone marrow by activated macrophages. When intestinal bleeding is present, the management is...

A 40-day-old baby girl presented with intermittent fever, lymphadenopathy, massive hepatosplenomegaly, progressive pancytopenia and features of disseminated intravascular coagulopathy. A bone marrow aspiration was performed and showed florid histiocytic proliferation with marked hemophagocytosis. Based on the diagnostic guideline for Hemophagocytic Lymphohistiocytosis proposed by the Familial H...

1 Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev 2004;3:69–75. 2 Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol 2002;14: 548–52. 3 Hibi S, Ikushima S, Fujiwara F et al. Serum and urine beta-2-microglobulin in hemophagocytic syndrome. Cancer 1995;75:1700–5. 4 Wellwood JM, Ellis BG, Price RG, Hammond K, Thompson AE, Jones NF. Urinary N-acetylbe...

Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues. HLH may be diagnosed in association with malignant, genetic, or autoimmune diseases but is also prominently linke...

Infection associated hemophagocytic syndrome is increasingly recognized as a potentially fatal complication of dengue fever. It should be suspected with prolonged fever beyond seven days associated with hepatosplenomegaly, hyperferritinemia, worsening cytopenias and development of multiorgan dysfunction. Surge of similar pro-inflammatory cytokines observed in dengue associated hemophagocytic sy...

Hemophagocytic syndrome (HPS) is a rare life-threatening condition caused by massive cytokine release from activated macrophages and lymphocytes. The paper depicts the development of HPS in different infections, malignancies, and autoimmune diseases. It describes a clinical case of hemophagocytic syndrome in a 63-year-old female patient with adult-onset Still's disease and high fever accompanie...

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia and low fibrinogen. Hemophagocytosis in the bone marrow is a hallmark of this syndrome. Ba...

Patients with rheumatoid arthritis are at increased risk of hematological malignancies, especially when exposed to immunosuppressive therapy. The mechanisms of lymphomagenesis remain poorly understood but factors implicated include high disease activity, exposure to antitumoral necrosis factor medications, and Epstein-Barr virus infection. Lymphoid malignancies of T-cell origin are uncommon in ...

Diagnostic criteria for hemophagocytic lymphohistiocytosis should be reviewed early in critically ill patients with toxic epidermal necrolysis, multisystem dysfunction, and a deteriorating clinical trajectory.

This case report reminds the reader of the place of hemophagocytosis and the H-Score in the diagnosis of secondary hemophagocytic lymphohistiocytosis.