Systemic mastocytosis presenting with a prominent B lymphocyte proliferation in the bone marrow and extensive fibrosis of the spleen

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dc.contributor.authorNese, Nalan
dc.contributor.authorCagirgan, Seckin
dc.contributor.authorErtan, Yesim
dc.contributor.authorSonmez, Ayhan
dc.contributor.authorSoydan, Saliha
dc.contributor.authorHekimgil, Mine
dc.date.accessioned2019-10-27T19:37:25Z
dc.date.available2019-10-27T19:37:25Z
dc.date.issued2007
dc.departmentEge Üniversitesien_US
dc.description.abstractSystemic mastocytosis is a disease characterized by multifocal mast cell proliferation in the bone marrow or other extracutaneous organs. Because of loosely scattered and hypo-/agranular mast cells, the diagnosis is sometimes very difficult. In the bone marrow, mast cell infiltration may be associated with prominent lymphoid infiltration leading to a misdiagnosis of a low grade non-Hodgkin lymphoma. A 49-year-old woman presented with right arm and leg pain, psychiatric symptoms, and diarrhea for four years. Physical examination and laboratory investigation revealed hepatosplenomegaly, anemia, mild thrombocytosis, mild leucocytosis and lymphacytosis. In the bone marrow biopsy, there was a prominent B lymphocyte proliferation reminiscent of a low grade non-Hodgkin lymphoma/leukemia and there were some spindle cells aggregates in paratrabecular location. The consecutive bone marrow biopsies were similar to the first. The subsequent splenectomy specimen exhibited striking fibrosis. In the lymph node sections, there was marginal zone hyperplasia. Multifocal accumulations of mast cells were strongly positive with mast cell tryptase and CD117 on immunohistochemical staining, though no metachromasia was identified in Giemsa and Toluidine Blue stained aspirates and tissue sections, probably due to hypo-/agranulation of mast cells. The case was presented to emphasize the importance of the antibody to mast cell tryptase in the diagnosis of mastocytosis and to discuss problems of differential diagnosis of systemic mastocytosis.en_US
dc.identifier.endpage171en_US
dc.identifier.issn0278-0240
dc.identifier.issue3en_US
dc.identifier.pmid17473386en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage167en_US
dc.identifier.urihttps://hdl.handle.net/11454/39971
dc.identifier.volume23en_US
dc.identifier.wosWOS:000246827600005en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherIos Pressen_US
dc.relation.ispartofDisease Markersen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectsystemic mastocytosisen_US
dc.subjectfibrosis of spleenen_US
dc.subjectmast cell tryptaseen_US
dc.subjectsmoldering varianten_US
dc.titleSystemic mastocytosis presenting with a prominent B lymphocyte proliferation in the bone marrow and extensive fibrosis of the spleenen_US
dc.typeArticleen_US

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