Gauchers disease is a rare lysosomal storage disorder characterized by abnormal accumulation of lipid-laden macrophages in different organs. Though hepatosplenomegaly is commonly found, symptomatic presentation with portal hypertension is rare. We report a child with liver cirrhosis and bleeding esophageal varices who was diagnosed with Gaucher's disease.

This case was of a 35-years old Egyptian lady, who works as primary school teacher, presented to the OPD with complaints persistent high fever for two days. Along this fever, she also had severe pain in multiple joints both hands, wrists, knees, and ankles. In addition, woman complained having developed rash on her legs, which so painful that unable stand without pain. The agonizing it prevente...

Introduction Macrophage activation syndrome (MAS) is a rare but life threatening complication of infectious, neoplastic and rheumatic diseases. Clinically, patients usually have high fever, hepatosplenomegaly, lymphadenopathy and neurologic symptoms. In laboratory results pathognomonic are pancytopenia, decline in the value of sedimentation rate (ESR), increased levels of ferritin and coagulati...

Hemophagocytic syndrome (HPS) is an uncommon manifestation in systemic lupus erythematosus (SLE). Clinical features of HPS include fever, pancytopenia, abnormal liver enzyme, hepatosplenomegaly, lymphadenopathy, and coagulation disorder. HPS comprises primary and reactive forms. Herein, we describe a case of untreated SLE with HPS as one of the first manifestations of systemic Lupus.

A syndrome of hepatosplenomegaly, thrombocytopenia, and anemia and the presence of sea-blue histiocytes in bone marrow has been associated with parenteral soybean oil administration in patients receiving long-term total parenteral nutrition (TPN). A case is described here where this syndrome was observed in a pediatric patient who received long-term parenteral fish oil nutrition.

The association between Hodgkins Lymphoma (HL) with HIV is common in adults but rare in children. A 5 year old boy, known case of HIV on antiretroviral therapy, presented with prolonged fever, multiple enlarged lymph nodes along with hepatosplenomegaly. A diagnosis of Hodgkins lymphoma was entertained on histopathological examination; further subtyping was done by immunohistochemistry.

Hemophagocytic lymphohistiocytosis is character-ized by fever, hepatosplenomegaly, cytopenia, hyper-triglyceridemia, hypofibrinogenemia, and hemophago-cytosis. Ascites is not mentioned as a symptom of hemophagocytic syndrome. We report a one month-old girl suffering from familial erythrophagocytic lympho-histiocytosis, who presented with ascites.