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.authorNeşe N.
dc.contributor.authorÇaǧirgan S.
dc.contributor.authorErtan Y.
dc.contributor.authorSönmez A.
dc.contributor.authorSoydan S.
dc.contributor.authorHekimgil M.
dc.date.accessioned2024-07-22T08:23:12Z
dc.date.available2024-07-22T08:23:12Z
dc.date.issued2007
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 lymphocytosis. 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. © 2007 - IOS Press and the authors. All rights reserved.
dc.identifier.DOI-ID10.1155/2007/960731
dc.identifier.issn02780240
dc.identifier.urihttp://akademikarsiv.cbu.edu.tr:4000/handle/123456789/19429
dc.language.isoEnglish
dc.publisherIOS Press
dc.rightsAll Open Access; Gold Open Access; Green Open Access
dc.subjectenzyme antibody
dc.subjectstem cell factor receptor
dc.subjecttolonium chloride
dc.subjecttryptase
dc.subjectadult
dc.subjectanemia
dc.subjectarm
dc.subjectarticle
dc.subjectaspiration biopsy
dc.subjectB lymphocyte
dc.subjectbone marrow
dc.subjectbone marrow biopsy
dc.subjectcase report
dc.subjectdiagnostic error
dc.subjectdiarrhea
dc.subjectdifferential diagnosis
dc.subjectfemale
dc.subjectfibrosis
dc.subjectGiemsa stain
dc.subjecthepatosplenomegaly
dc.subjecthuman
dc.subjecthuman tissue
dc.subjecthyperplasia
dc.subjectimmunohistochemistry
dc.subjectlaboratory test
dc.subjectleg
dc.subjectleukemia
dc.subjectleukocytosis
dc.subjectlimb pain
dc.subjectlymph node biopsy
dc.subjectlymphocyte proliferation
dc.subjectlymphocytosis
dc.subjectmast cell
dc.subjectmental disease
dc.subjectnonhodgkin lymphoma
dc.subjectphysical examination
dc.subjectpriority journal
dc.subjectspindle cell
dc.subjectspleen disease
dc.subjectspleen fibrosis
dc.subjectsplenectomy
dc.subjectsystemic mastocytosis
dc.subjectthrombocytosis
dc.subjecttissue section
dc.titleSystemic mastocytosis presenting with a prominent B lymphocyte proliferation in the bone marrow and extensive fibrosis of the spleen
dc.typeArticle

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