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Systemic mastocytosis (SM) and associated malignant bone marrow histiocytosis - a hitherto undescribed form of SM-AHNMD.

Rudzki, Zbigniew and Sotlar, Karl and Kudela, Andrzej and Starzak-Gwóźdź, Jolanta and Horny, Hans-Peter (2011) Systemic mastocytosis (SM) and associated malignant bone marrow histiocytosis - a hitherto undescribed form of SM-AHNMD. Polish journal of pathology : official journal of the Polish Society of Pathologists, 62 (2). pp. 101-4. ISSN 1233-9687. This article is accessible to all HEFT staff and students via NHS Evidence www.evidence.nhs.uk by using their HEFT Athens login IDs

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Official URL: http://www.termedia.pl/Systemic-mastocytosis-SM-an...

Abstract

An elderly woman presented with anaemia, thrombocytopenia and multifocal lytic and sclerotic bone lesions. Trephine bone marrow biopsy demonstrated widespread involvement by systemic mastocytosis (SM). The neoplastic mast cells expressed mast cell tryptase, CD117, CD25 and CD9, and were accompanied by compact sheets of atypical large histiocytic cells, expressing CD68, CD4, S-100 protein and CD14, in keeping with a concomitant histiocytosis (SM-AHNMD). Mutation analysis revealed the activating point mutation D816V of the c-kit proto-oncogene in microdissected pooled bone marrow mast cells. Partial remission was achieved using interferon alpha. To the best of our knowledge this is the first reported case of SM-AHNMD with histiocytosis as the non-mast cell component.

Item Type: Article
Additional Information: This article is accessible to all HEFT staff and students via NHS Evidence www.evidence.nhs.uk by using their HEFT Athens login IDs
Subjects: QY Clinical pathology
Divisions: Clinical Support > Pathology
Related URLs:
Depositing User: Mrs Caroline Tranter
Date Deposited: 25 Nov 2014 15:52
Last Modified: 25 Nov 2014 15:52
URI: http://www.repository.heartofengland.nhs.uk/id/eprint/668

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