IgM AL amyloidosis: delineating disease biology and outcomes with clinical, genomic and bone marrow morphological features
This study evaluates newly diagnosed IgM (6%, n = 75/1174) vs. non-IgM light chain amyloidosis patients. IgM amyloid patients had lower light chains (12.5 vs. 22.5 mg/dL; p 1 organ involvement (31% vs. 44%, p = 0.02) was less common in IgM amyloidosis, while soft tissue and peripheral nerve invol...
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Veröffentlicht in: | Leukemia 2020-05, Vol.34 (5), p.1373-1382 |
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Format: | Artikel |
Sprache: | eng |
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Zusammenfassung: | This study evaluates newly diagnosed IgM (6%,
n
= 75/1174) vs. non-IgM light chain amyloidosis patients. IgM amyloid patients had lower light chains (12.5 vs. 22.5 mg/dL;
p
1 organ involvement (31% vs. 44%,
p
= 0.02) was less common in IgM amyloidosis, while soft tissue and peripheral nerve involvement was more common. t(11;14) was less common (27% vs. 50%,
p
= 0.008) in IgM amyloidosis. Rates of
MYD88
L265P
and
CXCR4
WHIM
mutation in IgM amyloidosis were 58% (29/50) and 17% (8/46). Diagnosis after hematopathology review in IgM amyloidosis was pure plasma cell neoplasm (
PPCN
) in 23% (16/70), lymphoplasmacytic neoplasm (LPL) in 63% (44/70) patients, and other (14%).
LPL
vs.
PPCN
groups had distinct genetic abnormalities: t(11;14): 0% (0/18) vs. 60% (9/15),
p
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ISSN: | 0887-6924 1476-5551 |
DOI: | 10.1038/s41375-019-0667-6 |