Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the mononuclear phagocytic system, characterised by histiocyte and lymphocyte activation. It can be classified as primary secondary HLH. Primary HLH usually presents in childhood, associated with gene mutations. Secondary adulthood, due to an underlying infection, autoimmune disease or malignancy. We describe case acute hepatitis-A ...

Hemophagocytic syndrome (HPS) is a clinicopathologic entity characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. HPS may be primary, or secondary to malignancy, infections, auto-immune diseases and pharmacotherapy. In patients with adult-onset Still's disease (AOSD), HPS is a rare but life-threatening comp...

Fig. 1. (A–C) Features of the bone marrow (BM) aspirate and biopsy showing hemophagocytic lymphohistiocytosis (HLH) and cytomegalovirus (CMV) staining. (A, B) BM aspirate showing histiocytic hyperplasia and prominent hemophagocytosis by activated histiocytes. Arrowheads indicate features suggestive of ongoing endocytosis (Wright-Giemsa stain, ×1,000). (C) BM core biopsy result showing positive ...

A 3.5 month-old girl was admitted with silvery gray hair, light colored skin, recurrent diarrhea, chest infections, hepatosplenomegaly, episodes of pancytopenia, and hemophagocytosis in the bone marrow. Light microscopy of hair showed characteristic large and irregular clumps of melanin in the middle of hair shaft. Peripheral blood smear examination did not show giant granules in granulocytes. ...

The second son of consanguineous parents was repeatedly evaluated for failure to thrive, intermittent vomiting, diarrhea, hepatosplenomegaly and panzytopenia since his first year of life. Specific diets had no effect. Bone marrow aspiration showed hemophagocytosis, but no signs of malignancy. At the age of 6 years he developed a hemolytic uremic syndrome-like disease. Renal biopsy revealed mesa...

A 19-year-old boy with a 7-year history of extensive ulcerative colitis (UC), in remission for 4 years with azathioprine 2.5 mg/kg/d, was admitted reporting a 3-day history of fever (39– 40 C), headache, and abdominal discomfort. He denied other symptoms. Physical examination showed fever (40 C), jaundice, generalized lymphadenopathies (cervical, submandibular, occipital, pre-auricular, axillar...

Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here,...