{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,23]],"date-time":"2025-12-23T18:48:27Z","timestamp":1766515707940},"reference-count":16,"publisher":"Oxford University Press (OUP)","issue":"764","license":[{"start":{"date-parts":[[1989,6,1]],"date-time":"1989-06-01T00:00:00Z","timestamp":612662400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1989,6,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>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.<\/jats:p>","DOI":"10.1136\/pgmj.65.764.394","type":"journal-article","created":{"date-parts":[[2008,12,29]],"date-time":"2008-12-29T21:05:27Z","timestamp":1230584727000},"page":"394-396","source":"Crossref","is-referenced-by-count":17,"title":["Portal hypertension and ascites in systemic mastocytosis"],"prefix":"10.1093","volume":"65","author":[{"given":"M N","family":"Narayanan","sequence":"first","affiliation":[{"name":"Department of Clinical Haematology , Bury , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J A","family":"Liu Yin","sequence":"additional","affiliation":[{"name":"Department of Clinical Haematology , Bury , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Azzawi","sequence":"additional","affiliation":[{"name":"Department of Clinical Haematology , Bury , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T W","family":"Warnes","sequence":"additional","affiliation":[{"name":"Gastroenterology, The Royal Infirmary , Manchester, Bury , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W P G","family":"Turck","sequence":"additional","affiliation":[{"name":"Department of Medicine, Bury General Hospital , Bury , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1989,6,1]]},"reference":[{"key":"ref_1","article-title":"Zak (1957)3 no Bloom (1960)4 yes Capron (1978)1 no Grundfest","volume-title":"Sawers","volume":"no","year":"1980","unstructured":"Zak (1957)3 no Bloom (1960)4 yes Capron (1978)1 no Grundfest (1980)6 no Sawers (1982)"},{"key":"ref_2","article-title":"yes Yam","author":"Ghandur-Mnaymneth","year":"1985","unstructured":"Ghandur-Mnaymneth (1985)8 yes Yam (1986)9"},{"key":"ref_3","article-title":"Portal hypertension in SM was first reported in 1978.5 Since then 3 more cases of SM with portal hypertension have been described6-8 (Table I). In 3 other cases of SM with ascites, it is uncertain whether portal hypertension was present or not.349","unstructured":"Portal hypertension in SM was first reported in 1978.5 Since then 3 more cases of SM with portal hypertension have been described6-8 (Table I). In 3 other cases of SM with ascites, it is uncertain whether portal hypertension was present or not.349"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1111\/j.1365-2559.1979.tb03017.x","article-title":"Mast cells and mast cell neoplasia: a review","volume":"3","author":"Lennert, K.; Parwaresch, M.R.","year":"1979","unstructured":"Lennert, K. & Parwaresch, M.R. Mast cells and mast cell neoplasia: a review. Histopathology 1979, 3: 349-365.","journal-title":"Histopathology"},{"issue":"289","key":"ref_5","first-page":"119","article-title":"The spectrum of mastocytosis","volume":"1985","author":"Tharp, M.D.","unstructured":"Tharp, M.D. The spectrum of mastocytosis. Am J Med Sci 1985, 289: 119-132.","journal-title":"Am J Med Sci"},{"issue":"256","key":"ref_6","first-page":"56","article-title":"Osseous lesions in urticaria pigmentosa","volume":"1957","author":"Zak, F.G.; Covey, J.A.; Shodgrass, J.J.","unstructured":"Zak, F.G., Covey, J.A. & Shodgrass, J.J. Osseous lesions in urticaria pigmentosa. N Engl J Med 1957, 256: 56-62.","journal-title":"N Engl J Med"},{"issue":"168","key":"ref_7","first-page":"95","article-title":"Malignant systemic mast cell disease (mastocytoma) in man","volume":"1960","author":"Bloom, G.; Franzen, S.; Siren, M.","unstructured":"Bloom, G., Franzen, S. & Siren, M. Malignant systemic mast cell disease (mastocytoma) in man. 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Portal hypertension associated with systemic mastocytosis and splenomegaly. Gastroenterology 1980, 78: 370-373.","journal-title":"Gastroenterology"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1136\/jcp.35.6.617","article-title":"Systemic mastocytosis, myelofibrosis and portal hypertension","volume":"35","author":"Sawers, A.H.; Davson, J.; Braganza, J.; Geary, C.G.","year":"1982","unstructured":"Sawers, A.H., Davson, J., Braganza, J. & Geary, C.G. Systemic mastocytosis, myelofibrosis and portal hypertension. J Clin Pathol 1982, 35: 617-619.","journal-title":"J Clin Pathol"},{"issue":"109","key":"ref_11","first-page":"76","article-title":"Systemic mastocytosis with portal hypertension. Autopsy findings and ultrastructural study of the liver","volume":"1985","author":"Ghandur-Mnaymneth, L.; Gould, E.","unstructured":"Ghandur-Mnaymneth, L. & Gould, E. Systemic mastocytosis with portal hypertension. Autopsy findings and ultrastructural study of the liver. Arch Pathol Lab Med 1985, 109: 76-78.","journal-title":"Arch Pathol Lab Med"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/0002-9343(86)90622-4","article-title":"Hepatic involvement in systemic mast cell disease","volume":"80","author":"Yam, L.T.; Chan, C.H.; Li, C.Y.","year":"1986","unstructured":"Yam, L.T., Chan, C.H. & Li, C.Y. Hepatic involvement in systemic mast cell disease. Am J Med 1986, 80: 819-826.","journal-title":"Am J Med"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1002\/1097-0142(19820301)49:5<927::AID-CNCR2820490517>3.0.CO;2-B","article-title":"Systemic mast cell disease: a clinical and haematopathologic study of 26 cases","volume":"49","author":"Webb, T.A.; Li, C.Y.; Yam, L.T.","year":"1982","unstructured":"Webb, T.A., Li, C.Y. & Yam, L.T. Systemic mast cell disease: a clinical and haematopathologic study of 26 cases. Cancer 1982, 49: 927-938.","journal-title":"Cancer"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1111\/j.1365-2141.1975.tb00544.x","article-title":"Chronic myelomonocytic leukaemia","volume":"30","author":"Geary, C.G.; Catovsky, D.; Wiltshaw","year":"1975","unstructured":"Geary, C.G., Catovsky, D., Wiltshaw, et al. Chronic myelomonocytic leukaemia. Br J Haematol 1975, 30: 289-302.","journal-title":"Br J Haematol"},{"key":"ref_15","first-page":"339","article-title":"Chronic myelomonocytic leukaemia","volume":"67","author":"Skinnider, L.F.; Card, R.T.; Padmanabh, S.","year":"1977","unstructured":"Skinnider, L.F., Card, R.T. & Padmanabh, S. Chronic myelomonocytic leukaemia. Am J Clin Pathol 1977, 67: 339.","journal-title":"Am J Clin Pathol"},{"issue":"16","key":"ref_16","first-page":"808","article-title":"Bone marrow findings in systemic mastocytosis","volume":"1985","author":"Horny, H.P.Parwaresch; R., M.; Lennert, K.","unstructured":"Horny, H.P. Parwaresch, M.R. & Lennert, K. Bone marrow findings in systemic mastocytosis. 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