التفاصيل البيبلوغرافية
| العنوان: |
‘Occult’ mastocytosis with activating c-kit point mutation evolving into systemic mastocytosis associated with plasma cell myeloma and secondary amyloidosis. |
| المؤلفون: |
Sotlr, K., Saeger, W., Stellmacher, F., Stahmer, J., Jäckle, S., Valent, P., Horny, H.-P. |
| المصدر: |
Journal of Clinical Pathology. Aug2006, Vol. 59 Issue 8, p875-878. 4p. 1 Diagram. |
| مصطلحات موضوعية: |
*MAST cell disease, *CONNECTIVE tissue diseases, *IMMUNOLOGIC diseases, *MULTIPLE myeloma, *B cell lymphoma, *AMYLOIDOSIS, *LYMPHOPROLIFERATIVE disorders |
| مستخلص: |
A case of a 70-year-old man presenting with exsudative enteropathy due to light-chain-associated amyloidosis is reported. The diagnosis of systemic mastocytosis associated with IgG/λ plasma cell myeloma and secondary generalised amyloidosis was carried out by morphological evaluation of bone marrow biopsy. The c-kit point mutation D816Y was detected by molecular analysis. Two years before, a cystadenolymphoma of the left parotid gland had been removed. A moderate increase of loosely scattered spindle- shaped mast cells, a subpopulation of them expressing CD25, an antigen that is not expressed by normal or reactive mast cells, was shown by retrospective analysis carried out on art intraparotideal lymph node. The c-kit mutation D816Y was shown by the molecular analysis of the lymph node. In summary, the notion that systemic mastocytosis may very rarely be associated with B cell neoplasms and that neoplastic mast cell infiltrates may be obscured because of only a minimal increase of atypical mast cells, which are outnumbered by other non-neoplastic cells in the same tissue, is supported by this case. This finding was preliminarily termed ‘occult’ mastocytosis. [ABSTRACT FROM AUTHOR] |
| قاعدة البيانات: |
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