1/9. Portal hypertension and ascites in systemic mastocytosis.We report a case of systemic mastocytosis (SM) presenting as ascites and portal hypertension. The haematological picture at presentation was suggestive of chronic myelomonocytic leukaemia. Initial difficulties in making a diagnosis of SM were encountered as the cutaneous signs were atypical. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. The liver biopsy showed portal and sinusoidal mast cell infiltration, portal fibrosis and evidence of hepatic venous outflow obstruction. The disease progressed rapidly and recurrent massive ascites was a dominant problem. This case illustrates again the problems of making a diagnosis of SM especially when the clinical picture is atypical. ascites as a presenting manifestation of SM has been reported previously in only six patients. Published cases of SM with portal hypertension or ascites or both are reviewed.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/9. 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) |
3/9. Intractable ascites in systemic mastocytosis treated by portal diversion.A 50-year-old male presented with intractable ascites due to systemic mastocytosis. The diagnosis of systemic mastocytosis was established by histology of the bone marrow which showed mast cell infiltration and fibrosis. ascites was related to portal hypertension which was documented by esophageal varices at endoscopy and by an increase of wedged-free hepatic venous pressure gradient. Liver biopsy disclosed dense fibrosis of hepatic arterial and portal venule walls, resulting in complete obstruction of some portal radicles. peliosis hepatis and fibrous deposits in the walls of hepatic venules were also present. Because of intractable ascites and significant malnutrition, a portacaval shunt was performed which cleared ascites and dramatically improved the general condition of the patient.- - - - - - - - - - ranking = 1.2keywords = mastocytosis (Clic here for more details about this article) |
4/9. Systemic mastocytosis with portal hypertension. autopsy findings and ultrastructural study of the liver.Systemic mastocytosis is a rare cause of portal hypertension with only two previously reported cases to our knowledge. A 51-year-old man who was seen with intractable diarrhea and abdominal distention was found to have this combination at autopsy. At autopsy the liver showed bridging portal fibrosis. Subendothelial collagen deposition was demonstrated ultrastructurally. We present the possible mechanisms of portal hypertension in systemic mastocytosis.- - - - - - - - - - ranking = 1.2keywords = mastocytosis (Clic here for more details about this article) |
5/9. Portal hypertension in systemic mastocytosis.We report the case of a 66-year-old male patient with portal hypertension related to systemic mastocytosis. The liver was enlarged; microscopic examination showed portal mast cell infiltration and fibrosis. Portal hypertension was evidenced by splenomegaly, esophageal varices, and increased wedged-free hepatic venous pressure gradient. Arteriography showed that portal vein was patent. Portal hypertension could be the consequence of intrahepatic block due to mast cell infiltration and/or fibrosis of the liver.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
6/9. 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) |
7/9. Systemic mastocytosis, myelofibrosis and portal hypertension.A case of systemic mastocytosis is described in which the finding on initial presentation was hepatosplenomegaly. No dermatological abnormality was present, and the bone marrow histology originally caused some confusion with primary myelofibrosis. The clinical course and the importance of distinguishing between these two diseases is discussed. The dermatological manifestation of systemic mastocytosis, in the form of urticaria pigmentosa, is well recognised, and alerts the physician to the underlying disease. In the absence of cutaneous signs, however, the diagnosis is less obvious. The case reported had predominantly marrow and splenic involvement by the disease process, giving rise to portal hypertension, and illustrates the problems of diagnosis which can arise.- - - - - - - - - - ranking = 1.2keywords = mastocytosis (Clic here for more details about this article) |
8/9. A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent.Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis. CONCLUSION. Portal hypertension is a complication of systemic mastocytosis that can lead to death. Treatment with interferon alpha might be effective.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
9/9. Systemic mastocytosis: a rare cause of noncirrhotic portal hypertension simulating autoimmune cholangitis--report of four cases.Four patients with systemic mastocytosis, two men and two women, are presented. Three of them (patients 1, 2, and 4) developed portal hypertension and ascites without histological evidence of cirrhosis in liver biopsy. The remaining patient (patient 3) had severe bone lesions with multiple vertebral fractures. None of the patients had skin or lymph node involvement. Two patients (patients 1 and 2) died 12 and 9 months after diagnosis with acute nonlymphocytic leukemia and overt mastocytic leukemia, respectively, while the other two (patients 3 and 4) are alive 58 and 14 months after diagnosis. Treatment with hydroxyurea or cytosine arabinoside had not any beneficial effect in two patients, while a substantial amelioration of back pain had been obtained by local irradiation and recombinant human interferon-alpha-2b administration in one patient (patient 3). All patients had laboratory findings compatible with autoimmune cholangitis. We concluded that systemic mastocytosis is a rare cause of noncirrhotic portal hypertension often simulating autoimmune cholangitis and leading to the erroneous diagnosis of liver cirrhosis. diagnosis is based on the presence of mast cells in Giemsa-stained liver histological sections, and it may be confirmed by immunohistochemical detection of tryptase in the cytoplasm of these abnormally proliferating cells.- - - - - - - - - - ranking = 1.2keywords = mastocytosis (Clic here for more details about this article) |