Kostmann's Disease: A Case Report / 대한소아혈액종양학회지

Kostmann's disease is a rare hematologic disease entity which is characterized by persistent neutropenia and recurrent bacterial infections since early in infancy. The authors experienced a 2-month-old female infant with disseminated bacterial skin infection since 3 weeks of age and persistent neutropenia. Diagnosis was made by neutropenia in the peripheral blood, maturation arrest of myeloid hemopoiesis at myelocyte stage in the finding of bone marrow aspiration and improving response of neutropenia with G-CSF administration. She had experienced 4 episodes of sepsis and one episode of acute otitis externa during follow-up until 14 months of age.

Erythroleukemia Related to Chronic Ebstein-Barr Virus Infection / 대한소아혈액종양학회지

It is well known that the Ebstein-Barr virus causes not only infectious mononucleosis in the most cases, but also occasionally causes a variety of hematologic disorders such as anemia (hemolytic anemia or aplastic anemia), thrombocytopenia, neutropenia and malignancies such as Burkitt lymphoma and nasopharyngeal carcinoma. Furthermore, recently it is has been reported that it causes leukemia even though very rarely. The authors report a case of erythroleukemia possibly related to chronic Ebstein-Barr virus infection in a 14-year-old boy with brief review of related literatures.

Congenital Giant Aneurysm of Pulmonary Artery-Associated with Ventricular Septal Defect and Pulmonary Stenosis : A Case Report

Aneurysm of the pulmonary artery is a rate entity. A neonate was seen with cyanosis and tachypnea. There was a grade 4/6 systolic murmur along the left sternal border. The chest X-ray showed a round mass shadow in the left parahilar region. Echocardiogram showed large Ventricular Septal Defect and mild Pulmonary Stenosis. The cardiac angiogram showed giant aneurysm of pulmonary artery. Surgical intervention was advised. However, the patient was discharged against operative plan. And the patient died two weeks later.