A 53-year-old man was admitted with pancytopenia, fever and splenomegaly. Biochemistry showed increased ferritin levels. Bone marrow examination revealed increased erythrocytic precursors (94.9%) and active hemophagocytosis. Pure erythroid leukemia with hemophagocytic syndrome (HPS) was diagnosed. Induction chemotherapy comprising idarubicin and cytarabine was administered and steroid pulse the...

INTRODUCTION Combined with massive lung aeration loss resulting from acute respiratory distress syndrome, hepatopulmonary syndrome, a liver-induced vascular lung disorder characterized by diffuse or localized dilated pulmonary capillaries, may induce hypoxaemia and death in patients with end-stage liver disease. METHODS The case of such a patient presenting with both disorders and in whom an ...

We describe the case of a 17- year old female who developed fatal haemophagocytic syndrome (HPS) one month following acute infection caused by Epstein-Barr virus (EBV). Despite initiation of treatment and reduction of EBV load, laboratory signs of HPS as severe cytopenia, hypofibrinogenemia, hyperferritinemia and hypertriglyceridemia persisted, and the patient died of multiorgan failure. HPS is...

Hydropericardium syndrome (HPS) agent was purified by sucrose gradient centrifugation. The polypeptides of HPS agent were separated through sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) and identified by known protein markers. Eight polypeptides having molecular weights ranging from 119.0 to 15.7 KDa were observed. The nucleic acid of HPS agent was extracted and determin...

Background Hyperplastic Polyposis Syndrome (HPS) is a rare disease characterized by multiple or large hyperplastic polyps and carries an approximately 40% lifetime colorectal cancer risk. Although a genetic basis has not been established, HPS is believed to be a heritable syndrome and is diagnosed by clinical criteria as set forth by the World Health Organization (WHO). Based on clinical experi...

RATIONALE Chronic active Epstein-Barr virus infection (CAEBV) associated with hemophagocytic syndrome (HPS) and extra-nodal natural killer (NK)/T-cell lymphoma (ENKL) is a rare life-threatening disorder. This disease is easily misdiagnosed because of its varied presentations. PATIENT CONCERNS An 18-year-old girl was admitted to our hospital with a history of edema in the lower limbs and inter...

Hepatopulmonary syndrome is defined as the clinical triad of advanced liver disease, arterial deoxygenation and intrapulmonary vascular dilatation. Its pathogenesis is not completely understood. Excessive pulmonary nitric oxide production seems to be one of the factors that contribute to the intrapulmonary vascular dilatation. Other mediators such as endothelin-1 and the heme oxygenase-1/carbon...