Portal hypertension associated with systemic mastocytosis and splenomegaly. 1980

S Grundfest, and A M Cooperman, and R Ferguson, and S Benjamin

An unusual case of systemic mastocytosis with splenomegaly, portal hypertension, and bleeding esophageal varices is presented. Arteriograms and liver biopsy suggested the mechanism of the portal hypertension was due to increased blood flow in the splenic vein, although splenic arteriovenous shunting secondary to histamine release and increased intrahepatic resistance secondary to mast cell infiltration might have played a role. The portal hypertension was relieved by splenectomy.

UI MeSH Term Description Entries
D006975 Hypertension, Portal Abnormal increase of resistance to blood flow within the hepatic PORTAL SYSTEM, frequently seen in LIVER CIRRHOSIS and conditions with obstruction of the PORTAL VEIN. Cruveilhier-Baumgarten Disease,Cruveilhier-Baumgarten Syndrome,Cruveilhier Baumgarten Disease,Cruveilhier Baumgarten Syndrome,Disease, Cruveilhier-Baumgarten,Portal Hypertension,Portal Hypertensions,Syndrome, Cruveilhier-Baumgarten
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008198 Lymph Nodes They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system. Lymph Node,Node, Lymph,Nodes, Lymph
D008297 Male Males
D008407 Mast Cells Granulated cells that are found in almost all tissues, most abundantly in the skin and the gastrointestinal tract. Like the BASOPHILS, mast cells contain large amounts of HISTAMINE and HEPARIN. Unlike basophils, mast cells normally remain in the tissues and do not circulate in the blood. Mast cells, derived from the bone marrow stem cells, are regulated by the STEM CELL FACTOR. Basophils, Tissue,Basophil, Tissue,Cell, Mast,Cells, Mast,Mast Cell,Tissue Basophil,Tissue Basophils
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D004932 Esophageal and Gastric Varices Dilated blood vessels in the ESOPHAGUS or GASTRIC FUNDUS that shunt blood from the portal circulation (PORTAL SYSTEM) to the systemic venous circulation. Often they are observed in individuals with portal hypertension (HYPERTENSION, PORTAL). Esophageal Varices,Gastric Varices,Esophageal Varix,Gastric Varix,Varices, Esophageal,Varices, Gastric,Varix, Esophageal,Varix, Gastric
D006471 Gastrointestinal Hemorrhage Bleeding in any segment of the GASTROINTESTINAL TRACT from ESOPHAGUS to RECTUM. Hematochezia,Hemorrhage, Gastrointestinal,Gastrointestinal Hemorrhages,Hematochezias
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013154 Spleen An encapsulated lymphatic organ through which venous blood filters.

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