1/6. Pseudoxanthomatous mastocytosis.A case of xanthelasmoidea (pseudoxanthomatous mastocytosis) occurring in a 50-year-old Iranian man is described. The patient had a large upper gastrointestinal haemorrhage.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/6. Cutaneous mastocytosis with bleeding: probable heparin effect.We describe here a child with diffuse cutaneous mastocytosis whose early clinical course was complicated by recurrent episodes of severe gastrointestinal bleeding. These episodes were preceded by formation of cutaneous bullae and were associated with prolonged coagulation times. In the child reported here, it is likely that the degranulation of mast cells in the skin resulted in gastrointestinal bleeding by the dual actions of heparin producing anticoagulation and histamine producing increased vascular permeability. It is possible that our patient's symptoms were related both to increased number of mast cells and increased releasability of mediators from these cells; his clinical course suggested that an alteration of the latter occurred spontaneously over time.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
3/6. shock in an infant with bullous mastocytosis.A 6-month-old infant had bullous lesions on his posterior neck, upper trunk, and extremities for two months prior to admission for fever and shock. He had an elevated white blood cell count with left shift and normal platelet count, but abnormal coagulation studies. He was treated with intravenous antibiotics, crystalloids, fresh-frozen plasma, and pressor agents. A histamine H2 receptor antagonist was started for guaiac-positive nasogastric tube drainage. The patient recovered after four days of treatment. A skin biopsy confirmed mastocytosis. A week later the child passed grossly bloody stools with blood clots. No source of gastrointestinal bleeding was identified by extensive work-up. Blood histamine level measured one day before gastrointestinal bleeding was 16,400 pg/ml (normal 263 /- 202 pg/ml). The bleeding resolved spontaneously. The patient was maintained on cimetidine. Results of a subsequent bone scan were normal. shock or gastrointestinal bleeding associated with unusual skin lesions should alert the pediatrician to the possibility of mastocytosis.- - - - - - - - - - ranking = 1.2keywords = mastocytosis (Clic here for more details about this article) |
4/6. Systemic mastocytosis in a patient with polycythemia vera treated with radioactive phosphorus.Systemic mastocytosis occurred as a fatal event in a patient with long-standing polycythemia vera. The patient had been treated over the course of 21 yr with radioactive phosphorus. Possible relationships between mastocytosis and polycythemia vera, and also between mastocytosis and treatment with ionizing radiation, are discussed. Histopathologic and electron microscopic findings are illustrated. Difficulties in establishing the diagnosis of mast cell disease in this setting are also described.- - - - - - - - - - ranking = 1.4keywords = mastocytosis (Clic here for more details about this article) |
5/6. Variceal bleeding, hypersplenism, and systemic mastocytosis. Pathophysiology and management.Systemic mastocytosis is characterized by an abnormal proliferation of tissue mast cells. Though rarely a surgical disease, it occasionally presents as variceal bleeding secondary to portal hypertension. Ultrastructural studies of the liver and spleen and portal pressure measurements support the hypothesis that a perisinusoidal intrahepatic fibrosis is responsible for the increased portal pressure. When variceal bleeding complicates systemic mastocytosis, shunt surgery is indicated, with the type of shunt dictated by both hematologic and hemodynamic issues. Satisfactory blockade of histamine release can be achieved preoperatively by disodium cromoglycate and/or histamine antagonists to obviate any systemic effects precipitated by shunting of mast cell-rich splenic blood into the systemic circulation.- - - - - - - - - - ranking = 1.2keywords = mastocytosis (Clic here for more details about this article) |
6/6. Portal hypertension associated with systemic mastocytosis and splenomegaly.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.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |