Intravascular lymphoma is an aggressive and extremely rare extranodal lymphoma with neoplastic lymphoid cells confined exclusively within intravascular spaces. The histopathologic findings are subtle due to the rarity of the neoplastic cells in blood vessels. Clinical presentations are non-specific and focal space-occupying lesions or lymphoadenopathy are always lacking. It is a diagnostic chal...

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is an aggressive hyperinflammatory condition characterized by prolonged fever, cytopenias and hepatosplenomegaly, as well as hemophagocytosis by activated, morphologically benign macrophages. HLH may be characterized into two forms, familial and secondary HLH. Familial HLH usually manifests in children with genetic...

epstein-barr virus (ebv) is a ubiquitous human -herpesvirus that infects about 95% of the adult population. the majority of primary infections occurs in early childhood and is generally subclinical; it can cause infectious mononucleosis (im), which is usually a self-limiting lymphoproliferative disorder. however, infection of ebv occasionally results in severe, often lethal diseases, which inc...

Hemophagocytic lymphohistiocytosis (HLH) is the result of excessive cytokine release, leading to over-response by immune cells, such as macrophages and T lymphocytes. Here, we report a lethal case of HLH with a complete clinical course. The patient was a 45-year-old man with fever and chills since two months ago plus splenomegaly, hepatomegaly, and pancytopenia. The Anti-HBc IgM was positive, b...

Primary familial hemophagocytic lymphohistiocytosis (HLH; or familial erythrophagocytic lymphohistiocytosis [FEL]) is a heterogeneous autosomal recessive disorder more prevalent with parental consanguinity. There is aggressive proliferation of activated macrophages and histiocytes, which phagocytose red blood cells (RBCs), white blood cells (WBCs), and platelets, leading to anemia, neutropenia ...

Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No...

Hepatosplenic T-cell lymphoma (HSTCL) is a rare non-Hodgkin lymphoma, marked by liver, spleen, and bone marrow sinusoidal infiltration, with an aggressive clinical course, which represents a difficult diagnostic task for clinicians and pathologists. Another equally severe and challenging condition is the hemophagocytic lymphohistiocytosis (also called hemophagocytic syndrome [HS]), which is oft...

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is the manifestation of immune dysregulation. It is associated with ineffective but exaggerated immune response and infiltration of active lymphocytes and histiocytes in various organs. This devastating clinical condition has myriad of clinical and biochemical manifestations such as fever, splenomegaly, pancytopeni...

Salmonella infections are observed frequently in the childhood age group and mostly characterized with gastrointestinal findings. Extraintestinal involvement is observed rarely (8%) (1). Renal involvement has been defined in approximately 3% of the patients. Pyelonephritis, cystitis, hemolytic uremic syndrome, acute renal failure and rarely glomerulonephritis have been reported as urinary syste...

A 10-year-old male with a brain abscess developed pancytopenia, liver dysfunction, disseminated intravascular coagulation (DIC) and decrease of immunoglobulin A (IgA) level during postoperative antibiotic and anticonvulsant therapy. A bone marrow examination revealed hemophagocytosis. Real-time PCR revealed parvovirus B19 infection. The hemophagocytic syndrome resolved without specific treatmen...