introduction hemophagocytic lymphohistiocytosis (hlh) is a heterogeneous group of clinical syndromes characterized by activation and subsequent uncontrolled non-malignant proliferation of t-lymphocytes, histiocytes and macrophages leading to a cytokine storm and manifesting as prolonged fever, organomegaly, cytopenia, hyperferritinemia and demonstrable hemophagocytosis in the bone marrow. concl...

A 22-year-old man was admitted to our hospital because of fever, skin rash and epistaxis. Physical examination revealed fever (39.5°C), generalized purpura, lymphadenopathy and splenomegaly. Blood tests showed pancytopenia. Bone marrow aspiration and biopsy showed hemophagocytosis with no evidence of malignant cells. Anti rubella IgM antibody were positive and the IgG titers increased from 16 t...

www.paediatricstoday.com Visceral leishmaniasis (VL) is caused by the dissemination of the protozoan parasite Leishmania donovani throughout the reticuloendothelial system. This systemic disease may mimic or lead to several types of hematological disorders including hemophagocytic lymphohistiocytosis (HLH). Infection associated hemophagocytic syndrome caused by Leishmania is very rare and diffi...

Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH) encompasses an infrequent group of non-malignant, yet potentially life-threatening disorders caused by massive cytokine release from activated lymphocytes and macrophages (Filipovich, 2009; Henter et al., 1998, 2007; Janka et al., 1998; Janka, 2009). This multisystem inflammatory syndrome is associated with a ...

Dengue is a common acute viral febrile illness in the tropics. Although the usual presentation is that of a self-limiting illness, its complications are protean. We report a 29-year-old man who presented with an acute febrile illness and was diagnosed with dengue hemorrhagic fever. Despite appropriate supportive therapy, the patient initially improved, but subsequently had clinical deterioratio...

Chronic active Epstein-Barr virus infection manifests as a combination of persistent infectious mononucleosis-like symptoms and high viral load in apparently immunocompetent patients. It is closely related to Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. These 2 abnormal Epstein-Barr virus-associated diseases are seldom reported in individuals other than Japanese children an...

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), while uncommon, may be a devastating complication of lymphoma and/or human immunodeficiency virus (HIV) infection. While several of the diagnostic criteria for HLH are relatively nonspecific, particularly in the setting of a systemic inflammatory response, more diagnostic specificity may be achieved with marked elevations in serum ferritin (e.g., >100,0...

Histiocytic syndromes in children are divided into 3 classes: (i) Langerhans-cell histiocytosis (LCH) or Histiocytosis-X (HX), (ii) Hemophagocytic syndromes—Familial erythrophagocytic lymphohistiocytosis (FEL) and infection-associated hemophagocytic syndrome (IAHS) due to viruses (VAHS), and occasionally bacterial, fungal or parasitic, and (iii) Malignant histiocytic disorders(l). The lesional ...

Hemophagocytic syndrome is characterized by fever, fatigue, weight loss, lymphadenopathy, and laboratory abnormalities including pancytopenia, liver dysfunction, hypertriglyceridemia and hyperfibrinemia. Histopathologically, lesions are characterized by mononuclear cell infiltration with marked histiocyte proliferation and phagocytosis of erythrocytes, leukocytes, platelets and their precursors...