A Case of Idiopathic Myelofibrosis Causing an Esophageal Variceal Hemorrhage / 대한소화기내시경학회지

Idiopathic Myelofibrosis (IMF), a clonal disorder of a multipotent hemtopoietic progenitor cell of unknown etiology, is characterized by massive splenomegaly, leukoerythroblastic blood changes, and bone marrow fibrosis. Portal hypertension occurs in approximately 10% of patients with IMF. Alsom there mey be bleeding from esophageal varices. A 59-year-old woman was admitted with intermittent abdominal distension and melena. An esophagogastroduodenoscopy revealed extensive esophageal varices with red colored signs, and subsequently esophageal variceal ligation was performed. Marked splenomegaly and ascites was observed through abdominal US and MRI scan. Bone marrow biopsy revealed an increase in reticulin and fibrosis. Extramedullary hematopoiesis and sinusoidal change were observed in the liver biopsy specimen. This case of IMF causing an esophageal variceal hemorrhage is herein reported.

A Case of Esophageal Granular Cell Tumor Which Treated by 'O'-Type Band Ligation and Polypectomy / 대한소화기내시경학회지

Granular cell tumors involving the esophagus are rare tumors. Since Abrikossoff first reported five cases of granular tumor of the tongue in 1926, fewer than 200 cases of esophageal granular cell tumors have been reported in the world. The granular cell tumors usually were seen in tongue, skin & breast. it has rarely been noted in the esophagus. The treatment of this tumor was not established well. Therefore, we report a case of granular cell tumor of the low esophagus, which was resected by polypectomy following 'O'-band ligation. This 56-year-old male patient was transferred to our hospital because of abnormal endoscopic finding on the esophagus at local clinic. There was esophageal polypoid lesion on the low esophagus. After polypectomy, the lesion was revealed as granular cell tumor in histopathologic examination.

Kaposiform Hemangioendothelioma Complicated by Kasabach - Merritt Phenomenon with Bone Involvement in an Adult / 대한암학회지

Kasabach-Merritt phenomenon does not occur with common hemangioma, rather it is associated with the more aggressive Kaposiform hemangioendothelioma and rarely with other vascular neoplasm. We report the case of an adult who was diagnosed as Kaposiform hemangioendothelioma complicated by Kasabach-Memtt phenomenon. This is the first report in Korea of an adult with Kasabach-Merritt phenomenon who has osteolytic changes of femur, pelvic bone, and lumbar spine.

Persistent Truncus Arteriosus with Survival to the Age of 29 Years

The Truncus arteriosus is a congenital malformation in which only one great artery arises from the base of the heart and gives origin to the systemic, pulmonary and coronary arteries proximal to the aortic arch. Pulmonary blood flow is governed by the size of the pulmonary arteries and the pulmonary vascular resistance. In infancy, pulmonary blood flow is usually excessive because pulmonary vascular resistance is not greatly increased. Thus, despite an obligatory admixture of systemic and pulmonary venous blood in the common trunk, only minimal cyanosis is present. Rarely pulmonary blood blood flow is restricted by hypoplastic or stenotic pulmonary arteries arising from the truncus. The prognosis in persistent truncus arteriosus is very poor. the median age of survival of the 94 patients with the disease reported up to 1962 was only five weeks. The longest survival reported is the case of the man described by Carr et al who lived to the age of 36 years and 2 months. We report the case of a man with persistent truncus arteriosus who lives to the age of 29 years.

A Case of Isolated ACTH Deficiency Accompanied by hyperprolactinemia / 대한내분비학회지

Isolated ACTH deficiency is a uncommon disorder causing secondary adrenocortical insufficiency. Less than 200 cases have been reported in the world. The major clinical manifestations are hypoglycemia, weight loss, hypotension, anemia, weakness, nausea, inability to excrete water load, and hyponatremia. A 56-year-old male was admitted because of weakness, fatigue, nausea and vomiting. He was pallor and not associated with hyperpigmentation. The basal plasma ACTH and cortisol levels were 8.30 pg/ml and 0.6 ug/dl. The serum cortisol did not response to rapid ACTH stimulation test. On combined pituitary stimulation test, the cortisol did not response to insulin-induced hypoglycemia. Other anterior pituitary hormones showed normal responses except elevated prolactin level. Six months after glucocorticoid replacement therapy, the elevated basal prolactin level returned to normal. Brain MRI did not show any anatomic abnormalities of the sellar and suprasella area. We report a case of isolated ACTH deficiency accompanied by hyperprolactineia, which respond to glucocorticoid replacement therapy.