Familial Hemophagocytic Lymphohistiocytosis

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticulendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytopenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of them showed markedly decreased phytohemagglutinin induced lymphocyte proliferation and reversed CD4/CD8 ratio (0.52) in flowcytometric lymphocyte subset analysis. Aspirate of bone marrow revealed typical features consistent with FHL in two of them. In spite of recent therapeutic approaches, none of them survived.

A Case of Idiopathic Hypereosinophilic Syndrome

The idiopathic hypereosinophilic syndrome (HES) represents a heterogenous group of disorder characterized by prolonged eosinophilia of undetectable cause and multiorgan system dysfunction. Bone marrow is the most frequentry involved organ, but the most severe clinicopathologic involvement is heart. The major cause of death in patients with the HES is cardiac dysfunction especially congestive heart failure resulted from endocardial fibrosis and restrictive cardiomyopathy. We have experienced a case of DES with both cardiac and pulmonary involvements. The patient was an 18-month-old infant with poorly controlled lung abscess who complained of fever and productive cough. On the microscopic examination of pleural effusion, eosinophilic infiltration was noted. M-mode echocardiogram showed diffuse thickening of interventricular septum and left ventricular posterior wall. On the peripheral blood smear, prolonged eosinophilia was observed. We confirmed this case with bone marrow aspiration which showed eosinophilic hyperplasia.

Clinical Observation for Prematurity and Low Birth Weight Infant

No abstract available.