1/7. Slowly progressive systemic mastocytosis with high mast-cell burden and no evidence of a non-mast-cell hematologic disorder: an example of a smoldering case?A 43-year-old man with extensive systemic mastocytosis with poor prognostic indicators but no overt hematologic abnormality is described. This patient's clinical presentation and course are consistent with the newly proposed 'smoldering mastocytosis' category. Long-term follow-up of patients is needed to determine whether they may be at higher risk for progression into more aggressive categories.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/7. 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 = 1.1666666666667keywords = mastocytosis (Clic here for more details about this article) |
3/7. 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 = 1keywords = mastocytosis (Clic here for more details about this article) |
4/7. Systemic mastocytosis: a case report. Cytological, cytochemical and ultrastructural considerations.A case of systemic mastocytosis with unusual clinical manifestations, appearing as an isolated splenohepatomegaly, is described. The proliferative character is evident from the cytological characteristics of immaturity and the presence of a moderate percentage of mast cells in the peripheral blood. These cells make up 40% of the total cells in the bone marrow. Special attention is given to the optical, morphological, cytochemical, and ultrastructural studies of the disease. Some anomalies were found at the subcellular level which apparetnly have not been recorded until present. Various dyshematopoietic features of this case are reported, which may be considered as manifestations of a paraneoplastic syndrome.- - - - - - - - - - ranking = 0.83333333333333keywords = mastocytosis (Clic here for more details about this article) |
5/7. 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 = 0.83333333333333keywords = mastocytosis (Clic here for more details about this article) |
6/7. Systemic mastocytosis with review of gastrointestinal manifestations.A 63-year-old man presented with fever, splenomegaly, steatorrhea, diarrhea, and weight loss. A tissue diagnosis of systemic mastocytosis was made. This case is unusual in that diarrhea and steatorrhea were present in the absence of skin lesions and because fever was a prominent symptom. Thus, systemic mastocytosis should be included in the differential diagnosis of intestinal malabsorption even when the skin shows no abnormalities. The gastrointestinal manifestations of systemic mastocytosis are reviewed.- - - - - - - - - - ranking = 1.1666666666667keywords = mastocytosis (Clic here for more details about this article) |
7/7. Systemic mastocytosis--central nervous system features and lymphographic demonstration of lymph node involvement.Systemic mastocytosis is uncommon. A patient is reported with many of the characteristic lesions of this disorder but with two features not before described--the occurrence of mixed pyramidal and neuromyopathic neurological signs and the demonstration of lymph nodes with abnormal architecture by lymphography.- - - - - - - - - - ranking = 0.83333333333333keywords = mastocytosis (Clic here for more details about this article) |