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• W2080040311 abstract "Feyler et al (2008) recently described a series of 10 cases of true IgM myeloma, highlighting distinct immunophenotypic and cytogenetic features that included a high proportion of cases with t(11;14) (5/8 patients) and deletion 13 (4/8 patients), indicating a high risk cytogenetic profile. We wish to add to this series 2 further cases of IgM myeloma with variant cytogenetic features. Interphase fluorescence in situ hybridization (FISH) was applied on bone marrow aspirates for detection of deletions, duplications or rearrangements of the following chromosomal regions: 3q27/3′BCL6, 5′BCL6, 11q23/ATM, 13q14/RB1, 17p13/p53, 18q21/3′MALT1, 5′MALT1, 21q22/D21S259, and 14q32/3′IGH, 5′IGH, covering the major loci of known prognostic significance in MM. A summary of clinical and laboratory findings is presented in Table I. Fish analysis of IGH rearrangement in a patient with a true IgM myeloma. (A) Two representative cell nuclei from initial FISH analysis showing one separation of 3′IGH (green signal) and 5′IGH (red signal) and one normal red/green fusion signal. This abnormality was found in 25% of the cells indicative for any IGH translocations. Additional FISH analyses excluded BCL6, CCND1, and MALT1 as potential translocation partners. (B) Three representative cell nuclei showing normal IGH fusion signals on both chromosomes 14. This FISH pattern was found in 94% of the bone marrow cells indicating a complete cytogenetic remission. In contrast to the data reported by Feyler et al (2008) no translocation t(11;14) could be detected in our patients. A CCND1, BCL6, and MALT1 negative IGH rearrangement was found in one and a combination of monosomy 13 and a p53 deletion in the other case, illustrating the complex genetics of this rare MM subentity. In our patients, hyperdiploidy was excluded by using multiple FISH assays. In true IgM MM, IGH translocations including either t(11;14) or alternative aberrations are apparently a cytogenetic hallmark in the vast majority of patients. The favourable short-term treatment response of our patients to the bortezomib + dexamethasone (BD) regimen appears promising and favours the use of a BD-based chemotherapy, such as the protocol reported by Richardson et al (2005), in this putative high-risk situation. However, further clinical experience in this orphan MM subtype is necessary to confirm these preliminary findings. Dr W Willenbacher has received minor honoraria for lectures (<3000 EU) and an unrestricted research grant (regarding the sponsoring of the AUSTRIAN MYELOMA REGISTRY) of 30 000 EU by Janssen-Cilag GmBH, Austria, the manufacturer of Bortezomib." @default.
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• W2080040311 date "2008-09-05" @default.
• W2080040311 modified "2023-10-17" @default.
• W2080040311 title "IgM myeloma: more on a rare entity" @default.
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• W2080040311 doi "https://doi.org/10.1111/j.1365-2141.2008.07313.x" @default.
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